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LANGE medical book


1988

HARPER'S BIOCHEMISTRY
Twenty-first Edition
Robert

. Murray, D,

PhD

Professor of Biochemistry
University of Toronto

Daryl

. Granner,

MD

Professor and Chairman


Department of Molecular .Physiology and Biophysics
Professor of Medicine
Vanderbilt University Nashville,
Tennessee

Peter A.Mayes, PhD, DSc


Reader in Biochemistry
Royal Veterinary College
University of London

Vlctor W. Rodwell, PhD


Professor of Biochemistry
Purdue University West Lafayette,
Indiana

APPLETON & LANGE


Norwalk, Connecticut/San Mateo, California

.
.

2-


. .-. . .
. .


- . . .

1993

577.2

20.070

63

: ., ., ., .

63

: 2- . . 1. . .: - .:
,

1993. - 384 ., .
ISBN 5-03-001774-7

.
21-,

.
.
2- . :
, ,

, .
, , .

20.070

ISBN 5-03-001774-7
ISBN 5-03-001773-9
ISBN 0-8385-3648-4

(.)

(r.)

1988 Appleton & Lange,


Pblishing Division of Prentice
,
.., 1993

..,

.
;


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).
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;
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., . .

, . .
,
.

PYCCKtHIY ltIll

, .

1,
.
, KOTopLte
.
. . u.

: . , ,
,

(1981);

(1980);

. , ,

, , (1985).-u.. .

. .,

(1984-5);

,
, .

,
-

21-M


: , ,
.
.

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, .

. .

1 (<< )

.
. . ~ (<<
) ,
, , .

, , ~i
, .

,
.

(.

16)

~HK (.

36)

, (.

57),

,
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.

.

u()ue

,
. , BB~Y , .

( . 43).
,
( . 54).

( .

53).

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, .

11

.
.
.

111 .

, .

IV
-+ -+ .
, .
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.'
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lf, , , , ,
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,

1987

...
...
...

...

. . . , ,

, ,

1)

2)

!- ~
.


()

, ,

, .

1. 1.

.
~

,
,
,

(
) .

1.1. ,
.
.

11

1.

: ,

:,
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2.

;
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.

..

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,

4.

, , ri

,
,

5.
6.

-. ,

: ,

:
.

: ,

: ,

7.

: ,


11 Robbins S_ L., Cotram R. S., Kumar V.: Pathologic Basis of Disease, 3rd ed. Saunders, 1984,

, .

.1

10

1)

; 2)
; 3)

; 4) ;
5) .

. , ,

.
,

(
, ,

.).

, (

bls-),- , , ,

1.

, ,

. ,

, :

(),

. -

,

2.

,
( ),

30 :

7

.
,

.
,

- .

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1.

3.

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~ .

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, -

1I

. m

11


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1.

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(, ).

2.

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. Of'R,
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1900- .

n,

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2.1.

,
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2.1.

( ) 11

50
20
10
8,5
4
2,5

3rd ed.

m,

,
(Kt, Na+, ' Mg2+),

(Fe2 +),
I
0,8
0,4
0,4
0,1
0,01
0,001
0,00005

West . S. Todd W. R.: Textbook


1961, Jl.

(1-).

11

Biochemis[ry,

.
,

2.2,

e.n).

, , , .

,
.
(
) -

13

u 1.1. . . ,
, , 'l( . ,
, , 'I

( )

; (

(, )

(-

.
.

) .

. , ,

I ,
(
).
;


d -
, .

2.2,

. ,
r,

1.3. ~
65 11

2)

II
9
1
40
4

17,0
13,8
1,5

61,6
6,1

11 Davidson S. D., Passmore R., Brock J. F.:


Nutrition and Dietetics, 5th ed. Churchill Livingstone, 1973, .

2)

: J:, ,

22,5%'.

. : 1)
; 2.)

,
;

3)

. .

, .

2.3.

. -

in vitro.

2.1

().

., ,

XIX

14

( ,

, ,

).

0-40

).

(,
. .), .

0,25

( ),
. . . Mg2+ ,

7,4

[]

0,05

, ; ,

. . (
)
.
(, , )
.

30

1.1.

l .

(, ),

, ,

, , ,

(.

2.4),- ,

( ) ,
.

. .

, ,

. ,

(.

().

, ,

2.2).

. .

, ,
.

( )

Ie

. I

( , ) ()
.

(, ) ,

68qa .4. JC l . .
,


- (.)

11

(. )

;-6-

( -450)
(,

r )

Na/K +-
5'-

11

(, ,

- 21

11

11 rp ,

. , , .

, Jl .

-a::u: . , ,
; , .
21
Jl .

(2)

~~~~", (1)

(3)

105 000 9
60

15000 9

'Im'. ,

.....

,I,
.. 'i~'~-
~

~

1.. .l\ .
(, ) ,
( ) (1). (
) ,

PIIc:.

uJI (ll , 1) R

(2).

, (

9 ) np
-- (3). , .
,
.

16

I'
,

.
-

; ,
, - (.

2.4).

2.5. lC
. JI

.
, ,

. ()

nI' ,

II (,
)

JI

n (. . n

(- )

) .
, ,


(). ,

(,
),

-UII

>

, .

2.4.

2.1

(. .

I'

- .

) III

~
m
.

, 8
,

(.

. ,

),

, 8 . 2.4.

(. ).

,

:

- &

iii ll.
m ,

ROM
(-):
()

1)

, pa~ee

?,-

(.

.
II II (
)

); 2)
; 3) JIIIx
(..

).

1.

. 8

2.

: -

. ,
,
. 2.6. ,

2.5

:
.

n.."

.... 1.6.

17


-, ,

,
.
3.


(,

npe.

, . )
-
(,
)

, ,

, m

. ,

-,


; ,

, .

. ,
, ,


.
~ .

1.7.

in vitro.

2.7

(), !l

1)
2)
3)
4)
5)

6)

(, )

(- )

(, )
(, )

(.
)
, . -
-

, ,

1.

2.

'(

rOMoreHaTa

1.
2.


( )

3.

.
. . .

, .

tlRtl}

18

in vitro

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,
.

, . 2.7 .
2.3. 1. "

, ),

(,

, ;

2.
in vitro

, -

,
. .

. ,

, ,

(,

. ,

( ,

) ,-

, ,

. .

2.3

in vivo. 4.

, , ,

( .

JIll).

, , ,

5.

; ,

(,

(,

. .).

, ~

!.

, 1

VIVO

~ , Jl ( ll .'J. ..).
t

Jl

tJl ~ Jl

, . ,

, 1

vltro

J.

Jl

Jl

2.3.

. .l u

. 108 110
. ~
f.laBHbIx .l , .

Un.llu l'xu.uue .",

19

RR ( ,
). 6.

,
,

, .



1930-x .
,
- .
,
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1

SN),

.
-


( ).

. ,

(
, . .).

, .
, -

-+ -+ .

,
.

2.8.

,
.

, ,

in vivo
in vitro

JJ8. 2.8. ,

. ,

).
,

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,

.
.
. ;

-
.
.

in vitro,

-.

,
. ,

tlll

14

32

, ,

3SS

, ,

125[

. ,

1311

, , :

20

. , ,

~,

; ,


, ,

.
.

; , ,

Freifelder D. Physical Biochemistry: Applications to Biochemistry and Molecular Biology, Freeman, 1982.
Fruton J. S. Molecules and Life: Historical Essays the Interplay of Chemistry and Biology, Wiley-Interscience, 1972.
Weinberg R. . molecu1es oflife, Sci. m. (Oct.), 1985,253,
48. ( :
.)


(, ,
),
,

.
,
,

[,

( )]

.

;

'

L--R.

, ,

~x -

, .
.

3.4

3.5.

L-- (.

20

,
,

3.2).

20

ffi

3.1).
300 ,
20 .

() (.

cTpaHceM .

-,

.
,


, (
) (

).-
,

: ,

. - ,

(.

30).


(.

3.4).

R-

, ,

- , .

22

, R - NH~.
,

--

NH 2

3.1.


(R-COO-).

a-m.

, ,

. 3.2,.

).

.
:

, , (

7,0)

,
L-r:

L--.

=-log

3.1


, .

20

() (
)

. , ..
J .

-NH~.
, ,

R - NH j

, .
,
,

+,

R-NH~~R-NH2+H+.

R-

R-COOH +:t R-COO- +

(pl).

, . 3.3 ..

R-COO-

R-N 2 - (..

, .. ,

) .

R-

, R-N"- ,
R-COOH
, R - NH j.
,
(7,4 7,1 ),

R-COO-.

, ,

(R-COOH) = 2,35

pI


()

R - NH j.

I .

pI.

. .
2 (R-Nj)
9,69,

pI

(pI)

+ 2
2

2,35

+ 9,69
2

= 6,02.

I ,
, ,

, . 3.2,. ,
. 3.2.,
N +

. ,

~O-

, ,
.

- - -
2 10 (. 3.1).

, '

2
99%.

"

NH 2

~OH
11

. 3.2. .
, . .
,
; .
- ~.

.6

3.1.

23

--

2,1

ro- (, )

--

4,0 0,3

-I-- R

1_1

()

6,0

HN~N

HN + . NH

9,8 1,0

- ()

10,5

R-N +

-{_}-

{ }

- ()

R-N-C~NH2

R-SH

R-S-

NH 2

()

(u)

NH
1 11
R-N-C-NH 2

1:+

. , ,

pI

3.4? ,

10,1

= 2,09

12)5

8,3

3,86 = 2,98.

, ,

lO

lO

( uIO JI
.

3.4).

(), ..

, .

, ,

, ( . 3.4,). l-

}:11

. 3.3. -:)l: ).
,
. l:l:
.

pI =

2
pI = 9,7, pI = 10,8.
pI .
-
, pI

,-

.
lO lO .

, :-.

24

130} ...

~OH

I 29
(- ) ~

NH.+

IHO/ \\

pKz - 3,86

NH +

\\

(IJ-)

~s~

= 9,82

(-NH] .)

~~
NH!

\\

~ 6-8

3
=

(..( 1)

[3
!

\\ 0-

-() \\

(\

= -1

(>

11)
= -2

= +1

3.4.

:I "" .

. ,

(.

. '

F 7,0,
I
6
, pI = 8 - .

3.4)

20

D-.. D-
,

; D-
.


, ,
,
R-,
,-

(.

. (

3.2).

, )

. 3.3
,
.

(, , ):

(,

(> 2000 ).

. ,
.

).

( ),
(.

3.4

3.5).

, , -

, .. ,
.

.1

3.2.

JIc.tll L.,.u-n, ,

. <l ..1

R-rpynn

BeRe

(. 3.5)
-
2 , N ,

, .

ll

1'

11

~C)(OH
~c
11

3.5.

3.3.

L--,:lO. ' 11

fl

~KpaLeHHoe

Gly(G)

Ala(A)

Val(V)

'-2- --

Leu(L)

'"'

.NH

,2

--- "

Ile(I)

--

+NH:J

cu ( )

Ser(S)

-.

Thr(T)

'- :

+N

Tyr(Y)

.
2 )

Cys(C)

Met(M)

,-

s-

. KUCJIbIe

lC~

Asp(D)

Asn(N)

- 2 - --

--

I -

+N

2 "

11 (l) (), lCfl


. (. . 29 54). .

flfl .
2)

u. :

NH)f-

---z-S-S-Cz--C--

NH]+

26

fl

Glu(E)

Gln(Q)

Apr

Arg(R)

Lys(K)

H-N-CH 2-CH 2-CH 2

C=NH
I + 2
NH 2
- - - 2

His(H)

+N

_.
+ ...
'.&

..

HH~.

His(H)

Phe(F)

Tyr(Y)

Trp(W)

Pro(P)

,..

..

3.4.

-, .

u (.

(2--4-

29)

- -

(2--

I
I
S02 +N

3-
)


u (.

31)

2 - ---

(2--4-

+N

fH2-2-2-'- -.-

(2,5-

.N

+N

(.

31)

(.

31)

(2--5-

(.

31)


(.

31)

(.

(3,4--

32)

3-

-0-'2 --I

3,5-

3,5,3'-

()

(3,5,3',5'-

4)

+N

28

3.5.

, -

CH~H~OO

(3-)

(.

17)

+NH)

(.

(2-1)

2-2-1-

- ()

27)

(.

32)

+NH)
H)N+-CH~H-COO

(2--3-)

(.

,

570 .

35)

"'ma. =

+HN~

1 1 +

-,

1 .

~H20

,
2 , 2
-. N

+N~

, -.

( 4-)
.

3.6)

'-

YlYo-

, ;

(.

I-NH.

n_._

. ,

(AlaVal).

, yr .

3.7. (
).


,
, x
.

3.7). ,

(.

, -

.
.

II, ,


, yr
.

3.6.

29

R-bI , ,
R-

, ;

R-

. R-
"i

(cl.

. ,

R-

3.6).

= 7

(-SH)

, :r
.

--

,
.

.
,

. -

(.

3.9).


,
, , .

-

( , ).


, , ,

240 .

3.8,

l\

3.6.

\of

.
lC

,
l

,
,

I1 .,

OI"

xp~Ma

3.8.

ii",
-.

30

-= ~

L..

IV

>

10
~

Asp
Gly

,
.

Thr

~ ......

......

:.,

..

:.......

Cys

His
Arg

Ser
Glu

.,:...

".;. .:.:

3.9.

3..

s.'

- - -~
.

..

... :..

Lys

'

>.

IV

Ala

( ).

, .

Val


( , ,

).

Phe

Leu

, ,

.
(
). ,
,

,'=.....

Met

.'

;1.:.

-.~'

.: ......;.

:.~

"

. 3.10. ,
.
;

/ .'10 ( )

". ~

. .

.5%-

90-J 100).

(,

(Leu, II. , Trp. Val, Met.


Tyr) ,

Rr,

RA,a).

R r

(Pro, Ala, Gly)


(Thr. Glu. Ser. Arg, Asp. His, Lys, Cys) (. 3.10).

()

. ,

Leu)
,

(Gly, Ala, Val,

) .

. ,

. , ,

),

(.

Rr

900

3.11).

).

Rr
,
.

R r,

() -

.IIUm

./ / (4:1 :5)

... "8 . Asp

J
11

.GIU

Lys

,
.

.Ser

~spNH2'

.R

.Cys

31

il):Gly

.Thr
Ala

t GluNH 2

_ . Tyr

.Val
Arg J

Met ....

Ile Leu

... Trypt . . .
Pro
.

. ,

Phe

3.11.

, u

(
Anal. Chem. 1953:25:396).

N+-.

( )

~.

Na +.

. ,

(.

3.12).

s:

CD
~

1-

2
2

...

<{

::

s:

s:

::

1-

CJ

s:
s:

::

r:::: s:

ct

...s:

CJ

s:
'-

ID

s:

L.

<{

CJ

s:

s: U

s:

;s

:5:

::

ID

::

'-

:5:

::

1-

;s

'~

:r:

r::::

:
:

>

::

!.

:5:

s:

s:
's

;s

CJ

'1
411
~

!...

CJ
':5:

:5:

1-

;s

>

':5:

'-

::

s:

::

570

+ - - - - - - - -..~I

,25)<11.I-------___~~ ...- - -....

4,25 ....4f-----...

8. 3.12. ii 50 ( 550) . .. 6 Jl (5 ~ 0,9 );


JI 5,28, 60 . . Jl ii
. (55 0,9 ); Jl 3.25, ptl4,25.

. 6 .
- 180 . , Jl

570 440 . Jl 440 ,


( yt). ( . .

versity.)

Mertz, Purdue Uni-

32


, yr
(,
)
.


. 0,5-2.
2000-5000
.

6,4

- 1

. ,

, yr ,

, .
,
,

3,5

. .

, ,
.
.
,

Barrett G. . Chcrnistry and Biochernistry of the Arnino Acids,


Chaprnan and , 1985.
. G. Tools of Biochernistry, Wiley, 1977.
Friedman . Chernistry and Biochernisrty of the Sulfhydryl
Group in Arnino Acids. Peptides, and Proteins, Pergamon
Press, 1973.
Greenstein J. ., Winitz . Chernictry of the Arnino Acids,
3 vols, Wiley, 1961.
Heftman . Chrornatography: Laboratory Handbook of
Chrornatographic and Electrophoretic Methods, 3rd ed.,
Van Nostrand, 1975.
.
Touchstone J. . Practice of Thin Layer Chrornatography, Wiley-Interscience, 1978.
Zweig G., Sherma J. Handbook of Chromatography, 2 vols,
CRC Press, 1972.

.
,

.

,
, , ,

,- .

( , )

- (
.).


, ,

10

n.

.,

.1 .

4.1.

, N-OI (

-) ar

C-OI

)-.

- .

(, )

, ).

:- - .

) ,

'

, .

80-

(;

, ,

a-NH 2-

--

. -

4.1

. , n ,

.
2-1573

.
.

1.

N-
:

+ N

34

2.

'

Glu-Lys-(Ala, Gly, Tyr)-His-Ala

- :
.

11

+H,N

.,/

...........

"'"

t;"""

-IJ

/
N

. ;l ,

4.3.

lf .

/ N """ /

11

3.

R-

) - -
:

~


100

,
(

; . .

6).

.

Glu-Ala-Lys-Gly-Tyr-Ala


G
.

4.2.

.l 1

;\-
.l0 .
<1 N-It fJ.... yTaMaT (Glu. ). - <1Jl

(Ala,

).


()

.
7,4
u

.

tt,

- N-ubI

-
(.

J.I).

,
,

, (

, ).
-
, -

(..
t

- ).
N- , ,
( ' ),

()

100


.
,

,
.

3.3

(.

4.2).

, (.

.'1u

4.1.


(.

4.3).

;t.J ll .lII r-

Gly
Gly-Gly
Gly-Gly-Gly

4.1).

()

(N+)

2,34
3.12
3,26

9,60
8,17
7,91

nmu

(. .

). ,
,

.
,
,,

(.

5).

,
(,

) (.

35

45, 48).

5).

(.

-,

L-

, ,

S-

, D- IC)
.
.

(.

4.5).

N-

r
,

11 I

100

),

ZJ ;==\

CH z

, - 0-

11

C-N-<r--N

-
~Hz

NVNH

L-

-, .
( )

4.5.

, .
.

,
, :

(
).

l
.

I3- -


,-
,

Arg-Pro-Pro-Gly-Phe-Ser-Pro-Phe-Arg

(.

4.6).

Lys-Arg-Pro-Pro-Gly-Phe-Ser-Pro-Phe-Arg

(.

4.4)
N-

; ,

. , -

SH

11

.'1-

H--N +

.
2*

4.4.

3)

tH
~
./
"N
.
.
.
",/'
"-yH
T
I
~

(.

' z
CH

Hz

CH z

('-).

(
)

(30-100

) .

.
, ,

36
G

G QR

R N G

N G

N D

DLVAAEK

fj -

fj

'

. 4.6. ~-.'1. . 41 -58.


<(3-). . 61-91, .'1
J.

( 2 =

CHCONH 2)

, ,

(),

(<<) (



(.

1--4

4.7).

2-10%)

Ag+

(),

() . .

(),

. -

. .

4.8

r ,

. 4.7.

- ,
,

. d-,

37

nmu

t--

1.5

t-

aCB-2

+
' -1

aCB-1

..

..

.,-2

1.0 -

~CB-11

aCB-

~,

t-

N
N

.
..

::

'-1

80
70

Q.

90

l/l

11:

'-

50

40

ID

>-

I
I
I

.:

60

8.

::

J Jt

'-

200

400

600

20

800

10
10

20

. 4.7. - ()
.
-50. IO/o-
, 'l1 .

J. D. Pearson ! al., Department


stry, Purdue University.)

HCl

40

. 4.8.
: '

() .
- - . (
J. D. Pearson ct al., Department Biochemistry,

- - . (

1. ww .

oCb-

!.

0.51-

2~

' -2 1

.. ..i
i
..
Q.

Biochemi-

Purdue University.)

110

(,

).

}_

, ,

. r
.

, ,

(. .

. , q


Il, Il- Val)

50%.

20

96

Il-Il,

3.12)

Val-

24, 48, 72

(Val-Val,

, "


. .

, 96-
.

, ,

., Asx".

"Glx"

38

(.

14.9),

N- . ,

-2,4- (.

, .

38),

'

().

.
8 . 38, -

1967 .

N-
<"
. ,

, ,

~
. ,

CeHepa~

. ,



;
,
.
.

, ).
.

(.

N0 1

4.10).

OlN-V-_~ F + -~-CH -

t'HF

() ,

20---60



. 4.9.

I-2.4- ( ).
(1958 .)
.

.

( )
.

2,4r- .

- S- .

N-ubl
.

- (

30 100 ).

(CNBr),
CNBr.

-.

CNBr

- .

39

nu

'"

Trp-X.
.
. . '
JIj Asn-Gly,

NH

HN

0=

HN

)=0

~s

Staphylococcus aureus V8
Glu- ,
- . Glu-

NH

0-<

Lys .
CNBr-.
. Jl .
Asp"'Pro.
- ,

11

I!

Met, Trp, Arg

Asn-Gly,

'"
>0

Ji

NH

. -
,

0=

<

HN


(.

4.7),

(.

4.8)


-.

r
. 4.10.
(J3TeHCHa).
;I ii

(cleBa)

", l3- ()
. .

(
);

N-
( ;
. 4.11).
,

e
.

. .
.
,

( );

.
,

CNBr-

. -.
-

.
,

,
(

30-40
60 80

) . ~

'

N-

lC n. iI,
N-

i (. 4.11)
np . .

40

4.12.

...

-1

...
N-

en

Z,

JC

, ,
JC - , -.

, -,
,

.

J.l..

'N'

. . . . NHz

, ..

bl

1.

. 4.11.
( N- ).


.
, ,

. ,
N- , JC JC .

- ,
.

2.

. ;

,
.

4.

() .
, ,

5.

--

(,

).

(.

4.12).

3 .

(30

8
(21 ) 11

).

18 %;

(124

; .

5.10)

. -

nmu


, , ~
, , ,

, ,
.


L- ,
;

.
,

()
(.

45).

Cantor R., Schimmel . R. Biophysical Chemistry, Part 1:


The Conformation of Macromolecules, Freeman, 1980.
[ : ., ' .
.-: .

1984.]
Wold F. Separation of peptides phosph6cellulose and other ceIlulose ion exchangers. Methods Enzymol,
1977, 47, 204.
, . G. The Tools of Biochemistry, Wiley, 1977.
Craig L. , Cowburn D., Bleich . Methods of the study of
small polypeptide hormones and antibiotics in solution,
. Rev. Biochem., 1975. 44, 509.
Dayhojj . (ed.) Atlas ofProtein Sequence and Structure, Vol.
5, National Biomedical Research Foundation, Washington, , 1972, Suppl. 1. 1973; Suppl. 2, 1976; Suppl. 3,
1979.
Hash J. . (ed.) Antibiotics. I: Methods in Enzymology, Vol.
43, Academic Press. 1975.
Heftman . Chromatography: Laboratory Handbook of

Chin

. .,

41

Chromatographic and Electrophoretic Methods, 3rd ed.,


Nostrand, 1975.
Jauregu;-Ade/l J., Mart; J. Acidic cleavage of the aspartylprollne bond and the limitations of the reaction, Anal.
Biochem., 1975,69,468.
Mahoney W. , Hermodson . . High-yield cleavage of tryptophanyl peptide bonds o-iodosobenzoic acid, Biochemistry, 1979, 18, 3810.
Mahoney W. , Smith . ; Hermodson . . Fragmentation
of proteins with o-iodosobenzoic acid: Chemical mechanism and identification of o-iodosobenzoic acid as reactive contaminant that modifies tyrosyl residues. Biochemistry, 1981, 20. 443.
Marglin ., Merrifield R. . Chemical synthesis ofpeptides and
proteins, . Rev. Biochem .. 1970, 39, 841.
Needelman S. . (ed.) Protein Sequence Determination, Springer-Verlag, 1970.
Patthy L., Smith . L. Reversible modification of arginine residues: Application to sequence studies restriction of
tryptic hydrolysis to Iysine residues, J. Biol. Chem., 1975,
250,557.
Pearson J. D. et al. Reversed-phase supports for the resolution
of large denatured protein fragments. J. Chromatogr.,
1981, 207, 325.
Regnier . F., Gooding . High performance liquid chromatography of proteins, Anal. Biochem. 1980, 103. 1.
Snyder S. ., Innes R. . Peptide neurotransmitters. . Rev.
Biochem., 1979. 48, 755.
Stewart J. ., Young J. D. Solid Phase Peptide Synthcsis. Free. 1969.
Storm D. R., Rosenthal S., Swanson . . Polymyxin antibiotics, . Rev. Biochem., 1977, 46, 723.
Zweig G., Sherma J. Handbook of Chromatography, 2 vols,
CRC Press. 1972.

5
~

8000-1 000.

: , ,

(.

6), (. 54)
26), .

(.

, , ,

,
, ,

(.

56),

,
, 1907-1908 .
.
(. 5.1).

) .

.l

5.1.

ildl1 6C.1KOB. t'<l

:\10'-=

. n

70-80%- ,

; 60
()

,
.

J.I

Gly. Ala. Pro

43

:II: 11

. ,

. ,

, , ,

; ,

8,6
,-, 2 -,
-.

13-

(
),

, , ,

(. 26).
,

4.

3-

. ,

, , , ,

, .

1,


. . ,


t
, (.

). ,

. , ,

; ,

(.

5.2).

(),

, ,

(.

7).

,
,

, .
l

5.2.

tl

) (
, , . .
).

, . -

.1

L--

. ,
( .

. .),

}'

(),

(
()

tl

l),

(.

5.1).

, .

44

0-

11

.......... /
N

......... / ' N /
1

. 5.1.
;
C-N.

5.1

.
, .
(
S-S) J3- (
S-S
)
, ;

(. .

4.10).

().

tl

, , .

5.2, ,

,
,

1)

,
;

2)

;
,

3)

, .

, . .

(. . u-)

J3-).

( ),


,
.



,
. 5.2.
. ,
, .
-,
-R- .

, - -
, (
). ,
- ,
( ,

)

.

0,36 . "
( ). (

, ulig L., Corey R. , Branson . R.: The


structure of proteins. Two hydrogen-bonded helical figur
tions of the polypeptide chain. Proc. Natl. Acad. Sc;. USA
.1951:37:205.)

+ 1,

- 1.

,
,
.


(,

+ -)
,
,

u: n

45

"


, u

O,5r

,
1930- ., -

0,5--

. 5.4. - . R - ,
.
", , "

. (
Stryer L. Biochemistry, 2nd ed., Freemal1, 1981.
: . I:). 3- .- : . 1985.)

0,55

(.

5.2).

m ,
,

- (.

5.3).

R- -

(.

3,6

0,5-{),55

5.4).

0,54

. ,
,

0,15

. '(f (.

1.

-----1---

1.

5.5).

~
N~

2.

lr .54

(.6 )II)

-.

3.

0--

"
(
).

4.


,
.

5.
.

5.3.

"
-.

L-

, .

46

5.3.

Pro

Arg
Asp

Asn
Cys

Gly
Lys

His
Leu
Met

Ser
Thr

Irp

Val


),

N-

LI

- ( .

5.6).

- (
).

, ,
.

5.5.

---

-. - ~

R~ ~
~

( ). (
Haggis . . et l., Introduetion to
Molccul~r Biology. Wilcy. 1964.)


-,

- .

---

(
,

),
R-,

N-

- (.

5.3).

- - (
,
-).
-

, -
(.

5.6).

5.6. .l

-.

J'.

,10

NH-

. (R)
.
clTOMbI , - , -

. (
,

Stryer L. Biochemistry. 2nd cd . Free1981. .)

47


,
(.

4).

) ,
.

(-,
. S.7.
.n - /l. ~
Jl 124 .
.
-u-
, .
.

(.

8) POJ-4 (
Kartha G., J., Harker .: Tertiary structure ofribonuclcase. Nature 1967:213:862.) .

-)
(
).


,
,

. .

5.7

~-CTpYKTypa
. .
-, - (.

5.7).
-

, '

, Ll

~-CTpYKTypa

( ) ,

, .

5.7.

()
,

.
5.7,

.
,

,
, (

,
.

).
:

, - ,

- -.

- (.

16).

:lO

48

~':";~

()

.n .

( )

5.8.

8 (8)

l .

(
).


. ,
, .

5.8.

(-

) (

; . )

..

S-

S-

.
,

), (, -

. ), (Ag.

Hg)

( )-..

. ,

~. -,

(,

, ..
.

);

(. 42).

, ),

. .

.

UR
, Il


.

,

- ,

,
(.

(.

12),
23)

(.

18).

, .

.
,
.

,
, ~

(, )

; -

5.4. l1 -

(' .1

a-NH~,

,.

a-NH2
N-

Asp
Glu
Gly
His
Met

N-

N-

Ala
Asp

Ala
Asp

Gly

Gly

Gly

Met

Met

Met

__

---L.~---7_s_-s_J----I._2-0_21

11

I
- ---!L------1.L~-
{

. 5.9. - - , ~

Tyr
Val

_1 .

Ser
Thr

Ser
Thr

Val

, (.

Uy R., Wold F.: Posttranslational

covalent modification of proteins. Science: 1977: 198: 890,


(, .

4.10).

4.

}l

, .

5.4

5.5).

i;

Lys

N-

Val

-.


,
(.

5.1 ).

3.3,


(.

51).
124

Pro

-.

11

49

(.

- N- Gly,
- - Asn; - N- -

. . -

5.9).

Ala

, , ,

- - .

nPOll,

5.5.

lI lf.1

l(
-

- N

1 '
( )

N-

N-

Arg
His
Lys

Arg
Lys

N-

Uy R., Wold F.: Posttranslational


covalent modification of proteins. Sc;ence: 1977: 198: 890,
.

.t

00

..

... "

Jt

.,

..

'

Lys

Ser
Thr
Tyr
11

., .

48

5.10.

lO

. .'1

.l

:
.
~ .

N-. (

Smyth . G .. Stein W. . S.:


The sequence of amino acid residues il1 bovinc pancreatic ribonuclease. Revisions and conformaLions. J. 8;0/. Chem.
1963:238:227, .)

(l5

50

. .

.
( ),
.

l05g .
ll

) .

, .

\1 ,

20%~

. . . 105g.

.
.
.

"l

5.6.

~~ ,"' ~:\1()DI
()

<l

IIrOToMepa
- (L-: 2-",
. 2.6.1.1)

50000

2
2?'
22.
4

230000
29000
37000
33000

-- (-:2
[]. 6.4.1.3)

175000

() . (L-:

42.

35000

I-~.

(--I,6-

3.1.3.11)

(L-:2-
.

N- .

2.6.1.13)

1.1.1.27)

( -,

10

:!6000

12
22.
101.

48500
4100000
409000

3.6.1.3)
.

coli

(L-:

NH.

[].

6.3.1.2)

--Jl (- : 2 [],

6.4.1.2)

" Klotz 1. ., Langerman N.R., Darnall D. W.: Quaternary structure of enzymes. "". Rel ioc/lcm. 1970:39:25.
l

21

.IU:

51

( ). -

5-15%.

,
, .

(.

~-

), ,

100000

, ,
.

5.6

, -

Advances in Protein Chemistry, Academic Press. 1944- 1987.


(. )
Aisen .. Lis{m~'sky J. lron transport and storage proteins,
l. Rev. Biochem . 1980.49. 357.
Bald}~';n R. L. Intermcdiates in protein folding. . Rev.
ih .. 1975, 44,453.
Chou . ., Fassman G. D. Empirical predictions of protein
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Cro}; L. R. Handbook of Amino Acid Sequences of Proteins.
Joynson-Bruvvers Ltd. (Oxford. England), 1973.
Gurd F. N., Rothgeh . . Motions in proteins, Adv. Protein
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.
Hascllemeyer R. ., {leHarvell . Electron microscopy of enzymes. . Rev. Biochem., 1974. 43. 279.
Lennar= w. J. (ed.) The Biochemistry ofGlycoproteins and Proteoglycans, Plenum Press, 1980.
js ., Rossman . G. X-ray studies 01' protein interactions.
. Rev. Biochem .. 1974. 43, 475.
Neuratll ., Hill R. L. (ed.) Thc Proteins, 3rd ed., Academic
Press. 1975.
Osborne J. . Jr., ' . . Jr. Thc plasma lipoproteins,
Adv. Protcin Chem., 1977. 31, 253.
Smitll L. ., n . J., GOf{O . . Jr. The plasma liporroteins: Structure and metabolism. . Rc\'. Biochcm .
1978.47.751.

, ,

()

().
(.

6.1),

Q-

.

, .

, ,
,

. ,

, ,
(

~- )
(
,

).
,

- -

~-
.
(),

(V)

()

, : ,

V,

Pr, Pr,

(Fe2 +).

(.

V,

.'

6.2).


:
,

(Fe2 +

Fe3+),

(Mg2 +).

( ;
. .

12). ,
Fe1 +

reMo-

. ,

2,3-

()


.

. 6.1. . -
.
- ,
d, .

: ll

53

.
,

, ...

Rp (,

Leu, Val,

Phe, Met).

4,5

3,5

2,5 (. 6.3).

75%

-,

20

. N-,

. ,
, ,

0-

, , ,

. 6.2. . Ko.'Ibua ,
't" ,
;

QT N- . ,

F8 -

F,

His

. ',

(,

), ;

(Fe2 +).

, .
-
:

CTOPl;my ( ).

F8

() 7 () (.

6.3).
,



.
(,

)

, (
; . . 13).

Il

17000;

153

:
,

Rp;

, ,
(,

Thr, Trp ., Tyr),

, -

. 6.3. . -
, .
-: . (

Dickerson R. . In: The


. (editor). Academic Press,

Proteins, 2nd ed., Vo). 2. Neurath


1964, .)

54

6
n

His (F8)

,
,

ll

3,5

(, ),
- ,

Fe 2 +

) . . ,
,

,
n

His (7)

. .l
,

6.4.

. (

l' ,

. .

et al . Physiol6gische Chemie. Springcr-Verlag,

1975.

.)

F;

His F8

His F7.

(.

7)

6.4).

His F8.

His F8

Fe2 +.

(His

His

7 (.

6.4).

1210

(.

6.5).

~O

0,03
His F8.

0:;;.-'
__

I
Fe

111

_ _ Fe _ _

0,01

. ,

, ,

His F8

6.5.

yr.lepoJ1L1.

J1

( ).

: z

()

25000

100

80

lf/.

. ,

02?

::i
u

40

::r

20

(Fe,

60

s
~

:J

, )

(.

6.6).

55

80

40

2 ,.

100

. .


(.

6.6).

6.7.


,
,

200

, -

02'

1%)

(<< ),
.

(
, - ).

1 (

).
(.

[:5

~O

6.7).

[:5
~O

C~

40 . .,

,

. ,

, 02

.;
,

.

..

:
;
0 2 Ica

1) 02
2)

Fe

Fe

~)

.
2

, ?

, ,

- 20 . .
20 . .

IC

J 100 . .,

~)


,
,

6.6.

.
7
-
. 900 .

( , ~,y, ,

S . .).

6 "
- ~- -

-
, ~--146; - ~
()

141

~-, - -

lf/.

100
90
80
70

60

%
CII

:J 50

u
40

::r

20

20

40

80

60

100

120

140

, . .

,
,

~-
,

' . -
,
, .
,

, (-
)-
;

-,

~-.

. 6.8. l .1() :
~.10. Jl .l0
:l :\1 1. .
- .10 40 :\1:\1 . .,
ll - .10 20 . .; .1

, .1.1 Jll1
. ..., 5 . . 'J
. l0 II ll
J

.
. (l0J1
.1 .
:\1.10. ilHa.1or
.1 .) ( Stanhury J. ..
\Vyngaarden J. ., Fredrickson . S. (editors): The Metabolic

Gw-ilI.

Basis ol'lnherited Diseases. 4th ed.

1978,

.)

, .

: (

)-2~2;

)- 22;

HbS

HbF

)-2 S 2 ; 2 (
)- 2 2 .

(
),

02

02

Toro,

02'

02

~ 02 02'
.
, ,

= 100

. ., ,

= 20 . . (. 6.8).
2
02 -

.
( . - ,

so -

'

, ),

so

,
2

(HBF). so = 26 . . .,
HBF P so = 20 . .
F ,

);

, (. 6.8).
, -

HBF ;
02'

57

: ~f ll

A~iS~COO- ~2
94

O~

,
(.

6.9).

02'

.
,

0-

..

Arg

02

(.

6.1

6.11).

feMor

- (

40
126

/His
146

141

Asp
94

+
NH 3 /3,

/-,

NH+

~Asp__Arg. -

0-

. /-

Lys

14'---1~
Lys
/40

NH 3

10/

Asp

. 6.9.
. - .
.
. ( Stryer L.: Biochemistry, 2nd ed .
.)

Freeman. 1981.

. tut-);

(0 2 ) R- (re/d-) (. 6.12). R-

(F8),

,
,

0,06

(.

CO z

0 2

15%

0 2 , ,

02

(.

6.14).

- ,

6.13).

_/

J
I

J__

-- j --

....
I

\
T~opMa

11,

R -

. 6.10. - R-
r
( 2/ 2 ) 15 (u./P.). , . .
( 2/2) . u 2/ 2-

u,/.- ( )


(-ij)

F-i;....

'"'11

F8

~H

o~::::e;:O:eHJC_N\fe@

___

..
-

08oro

Tyr

",

7 42)

(R-)

. 6.11. . . d ul,f32-KOH .
) ,
.
. ( Perutz . F.: Molecular
p<lthology fhu hemoglobin_ Stereochemical interpretation
of l oxygen affinities_ Nalure 1971 :232:408, -

. 6.13.
,
_
F8_ ( Stryer L: Biochemistry, 2nd ed., Free.

1981,

.)

.)
-

R-

. 6.12. - R-
. .1 ;1 ( ),
. . . .
( "lII). - R-
<I"13 .

. , . .
IC. . .
.1 - R-
J1 , :. _ ( Perulz . F.: Hemoglobin strueture
and respiratory transporr. Sci. m_ [.] 1978:239:92, .)

: .'?1u ..lOUll

+ 2

&

ll

2
lRR

+====~.

+ +

59

.
.

dJIO

, ,
.

6.14.

l ,

llll - .

-;

CO~ (. 6.15).
l.
-
,
~
. .

( )

~-.

. ,

6.15).

,
.J -,

~-.

(.

(146)

R-

. .
, ':'

COZ

TeTpaMepHoro

-bI , -

20 2

).

2 2

2,-

2H 2 CO J

11

111r-17:~~:-:~:"J!':"::":a:::::-:;:-J-1
2HCO J - +

rBIoIAbIxaeMIoIM

2+

Hgb.40~2 402


2,- (

, ) (. 6.16).
l,-.

2+

402

+2HCO J '

.
,

Hgb.2H+

11

()

2 2

r'"'I"7"~~--.

.
,
-

\\

. b.15. . ,
ll , ,

"

.
-- l>l
,. l
.:.

o\~

-, 'J

} .

-"

( ) .

",
\\

"

0-

0-

6.16.

} 2.3-.

60

J3-.

,
J3-:
ValNAl, LysEF6
His Hf 1 (. 6.17).
. r

- ror, e

Il- -

- R-.
or
, ,

J3- 21

His,

Ser,

,
. -
-

r or

.
.

reMor

R-

r .


( 3000 /)
. ,
Fe2+


,
.


, - J3-,

), .


, .
,
,
l'.

ror

r or r - Il .

Fe3+ -,

, -

02' -
R - -
-. ,
. J3-
~ R- - ,
, .
,

R- (
), ,

.


.
, (
).

His

21

S Glu Ai6)J3

2 (Glu Val)

Val.

, Glu
l
J3- .

S (

). r
r ror

,
J3-,

(. 6.18). S
. 6.17.
.
~ P-u ..(
Amone .: X-ray diffrac110n study of bondl~g of 2.3diphosphoglycera1e 10 human dhmgl. Nature

1972:237: 146,

.)

,
\.:

. ror

. .

61

. 6.18. , S ( )
( ) S.
S .
, '
. ( Stryer L.: Biochemistry, 2nd cd., Freeman, 1981, .)

, .

. ,

S,
or

. ,

S.

), -


R- .

:

S)

S - (

R-.
I-

.
-.

S (. 6.18),
S

. , r

R- - S

S .

(.

6.19).

. 6~19.
~~ S. (

Maugh . 11:

new understanding of sickle 1l emerges.

Sc;ence 1981:211:265,

.)

62

6.20.

() 11 () lJ.. r

, )( '\.1 ]I. '3. ,l ~ 8 (


), ~)l> I1f ~-6 II '~t ~II (.1. 36).

:
(.

6.20),

-.

(-)

(-

). ,
.

,

(. .

36).

Dean J .. Schechter . N. Sickle-cell anemia: ll and l


ll basis of therapcutic approaches. (3 parts). N. Engl. J.
Med., 1978, 299. 752. 804, 863.
Klot;: 1. .. n D. N.. inK L. . Ra1ional approaches 10
chemotherapy: Antisickling agents. Science. 1981,213. 724.
Perut;: . F. Hemog10gin structure and respiratory transport.
Sci. m. (Dec.), 1978, 239, 92.
Perut;: . F. The rcgu1ation of oxygen-affinity of hemog10bin:
I of structure of globin heme iron, . Rev.
Biochem . 1979, 48. 327.
Stamatoyannopoulo.'t G. The mll1l basis of hemoglobin disease. . Rev. Genet., 1972. 6. 47.
Winslm~' R. . .4nderson /'. F. Thc hemoglobinopathies. Page
1666. In: The Metabolic Basis of Inhcrited Disease. 5th ed.,
Stanbury J. . et l. (eds.), Gw-iII. 1983.

:
.1

(), -; ,

,
aTc .

.
(+,

(),- .
.
, ,

. . . .
.

(IUB),

. ,

-,

. .
,

.
.

;
u

. ,
{J

l\tI

. ,


. (

.
(, , H~
)
.

(, ).

,

.
.

,
,

13).
2. :

- "f

( );

-.

3.

Jl .

L-

+ NADt

+ NADH + t,

L-:

1.1.1.37

NAD t

().

4.

IIOMep

():

-
.

--

IUB,

, ,

1.

(). ~; , -

2.7.1.1 ,
2 (). 7
1 (

).

. .

D--6-

64

.
.
.

3.1.1.8.

= + .

, JlLI

3.2.

. :

1.

. ,

S80CC

+ S'I<CJ1 =

S':

SI<CJ1

-,

-,

= ,

NH2 -CH-NH-. :
1.1. , ll

( ). :
1.1.1.1. : NAD+ [
]"

+ +.

CH-NH1

( ). :
L-:

NAD(P)+

() [
].

NAD(P)+

NAD+ ,

NADP+.

+ 2 + NAD(P)+ =
= - + NH"'4 + NAD(P)H + +.
2.

2 =

D-.

, .

11

, ,
,
,

N-.
,

3.4.21.

( )

S-G + S'

. :

IXa XIa.
3.4.23.

S':

= S'-G + S.

(c)) ..

: , .

4.

. ,

L-

, , ,

1.4.1.3.

[~

. :

+ NAD+ =

NADH
1.4.

~-- -

~-D-

3.4.

+ S'8.

3.2.1.23.

- = -

, -, -,
N, C-S -. :
4.1.2. -. :
4.1.2.7. -l-- []
-l-

= +
.

, ,
,

. :

4.2. - . :
4.2.1.2. L- - -: []

2.3. QJ. :
2.3.1.6. -: -
[-]
-

2.7.

5.

-.

, ,

. :

2.7.1.1.

: D- 6- [

3.

D- =

2.

.:

5.2. u-m-. :
5.2.1.3. -m- ll--m-
[]

+ D--6-.

. , m

, ,
, ,

, - P-N. :.
3.1. , LI
. :

L-

-m- = ll--.

5.3.

. :

5.3.1.1.

D--3---

[]
D--3- =
.

fn:

r. ( . -).

6.
.

0-

./

./

.........

11

. ,

C-S, C-N -.

-,

65

L-

6.3.

C-N. :
6.3.1.2. L-:

(ADP)

11

NAOH +

-: 2 () [

6.4.1.2.

0,/

7.1. -
, ro NADf- 8 ,

-. :
-

.........

L- + NH~ =
+ L-.

6.4.

>-<

NAO+

,/

+ - + 2 =

u--

, "
,

+P +
j

-.

(
)

. ,

NAOH

NAO+.

NAD+

(
,

NAOH

NAO+,

NAO+

, , ,

NAOH,
7.1).

(.

, (

IUB

1,2,5 6).

,
,

IUB

4).

7.1.

~ "Jii llltl

NAD

r.

, . .

, .

, -

,.

(.

7.1).


3 1573

co/i

66

D-G
,

D-G, .


( ),

(
).
:

D-GX
D

A-G

CoE-G

,
:

D-HX

D-HX

CoE-

34.1).

()

NAD~

..

(.

NADP+

7.2.

. ,

1.1),

(,

(.

, ,

COE
CoE-

G),

,
,

+ +:t A-G + .

6
B..L- lI, , Jl

, ,
). .

1.3

, _- :

11

GJ'NH'

CoASH

O---CH 1

~H

(B I2)

, :

NAD+, NADP+
FMN, FAD

=--

NH 1

N~N~
I
I 7
'N

O=P-O-C~H
I

. , , ,

. 7.1..

R (
--2- ( NADP+).

NAD(P)+;

NAD+)

.wll:

67

: (),

, 110

~'1

. ,

-, L-. :

(,

D-

L-
.

7.3.

H~

, .

(
),
,

1 3


: Il

,
( ~),

, ,
(
)

. ,

1 3,

II

, -

1, 3 ( ).

,l

L-,

, L-.

, ,

. ,

. ,

. .),

NADP+,

NAD+,

. , ,

, '

(,

),

NADPH,

, -

68

(,

1,0

),

NAD+.

..

\
I \
I \
I
I
I
,
I
,

...

.
:

0,8

. ,

0,6

: ,,
--' ,

11:

..
s

0,4

.....
/

-,

0,2

"

R
.

NADH

\
\

,,
"-

250

200

300

"
400

350

. ... ,

npo

. 7.4. NAD+ NADH. l1


44 /. I .
NADP+ NADPH .


,
.

.
(,

), (,

10-6

10-9 )

(NADH).
(, . -

(, 10-12 )
(
). (

.... U, nU

pU.

"
;

:::

,
NAD+ NADP+ (

0,85

),

NAD.f-

340

NADP+)

(.

NADH

NADPH

7.4).

NADH

0.80

NAD+

340 ,
D
(. 7.5).
(. 7.6)
(D)

1,0

2,0

..,

( .

7.5)
.

,
J.I D 340 .

. 7.5. NADH-
ND- . r
340 .

. (S).

.l0 340 .
l- 10 NADH (J1
NADPH). 0.025- 0.2 .OJ- 80 .

_wu:

69

~I


, ,

.
,

, ,
,

0,05

0,10

0,15

0,20

... ,

. lle

. 7.6.
_ !IO
, _ 7.5, ;I _

.. -

.

)
,

.
lll
,

(.

7.7).

I( MeTO~

:

(
), (
, );

lll

~TP, Mg2+
~DP,M92+

lll

6-Il

, .
, , (
)

-6- II

. lll

NADPH+ +


6-Il

. 7.7. ,

,

-6- .

-6-, , , Mg2 +
NADpt . " ..l0

:I . _1 -

NADPH,

340

lll 490-
. 7.2.

.
,
( 1
) llM
.

70

7.2.

pU

Mr

pU/Mr

100000

10000

10

(100)

98000

8000

12.2

98

90000

1500

60

90

60000

250

240

60

58000

29

2000

58

52000
50000
49000

20
12
10

2600
4160
4900

52
50
49

100000 g

, 40---50%-

(NH4)2S04
, 20---35%-

80---11

-
, 43-48%-

(NS.

wl

, d

,
.


~
,
, .



,

,

,
() 3-8 .

NAD+

NAD ~

, ,

,
.


,
Q (,
),
, -

, <? , I<


(, )
.

.

,

(NH4)2

S04~

u
.

.
-

ll (. ).

. -

: ('n

71

, . .

. ( ')
I (

) ,

(<<)

, ,
,

).

, .

ll

>, .

(. .

2) .

, , , ,

(. .

2).

in situ

(<<).
(

10



, ,

. ,

~~

8,6

()

-6-,

-6-

( , . /

, . ,
,

.
,

1)
2)
3)

(, );

(NAD+);

(,

);

4)

NADH

[, ()];

5)

; ( ).

NAD+

72

t.H

./

........ / '

0-

r----=-----,

HJC

~
/.........

L- ,,",

0-

/'

,
:

""",,

PIIC. 7.8.

NADH +

NAD+

L--

(.

7.8).

1.

Is

37 ,

\ (.

7.9).

1,

Is,


7.10).

(.

12'

14.

1,.

1,
12
1)
14

Is

,
, MorYT
.

- -

' (, )

).

(,

, .


.
rr (.

130000)

, (

34000).

()

SH

~~'~

()

NADH + +

NAD+

7.9.

, , It.
,

.
,


( )

Jl

perpdMMC

()

>---==<

, ,

>--=<

. ,

llm: ('ROlil"mflll

73

. 7.10.
() .

8,6'
, .

. .

. . . .
u . (
Dr. Melvin Black and Mr. Hugh Millcr, St Luke's

Hospital, San Francisco.)


5


. ,

,-

. .

7.3 ,

, ,
,
,

u ,
.

.
,

. ,

(Southern, 1975) -

74
.-.
8

7.3. .

l0
l: ,

il ,

-,
u.-

l., 1983).

(Kidd et

(. .
,

38).

(l),

um

(Val);

~-
,

11

Sau 1 (Orkin et

l.,

Mst

1982).

ee~

( ),

().

).

( ,
). ,

, ,


. ,
(

; . .

1), ~- (
Hind 111 1) (Little et
l . 1980; Woo et l., 1983).

6)

(Woo et l., 1983)


(. . 31). , , .
,
,

~- ~,
(Dozy, Forman, l0, 1979; ,
Chang, Dozy, 1982).

,
,

He' .

, ~-


,
(Cavenee et l., 1983)
(GuseIla et l., 1984).

(Pirastu et l., 1984).


u.- - ~ -
.

u.- -


. 36.

",:

ll II

D.. Lardy ., Myrhiick . (eds.) The Enzymes, ..d ed.,


7 vols. Academic P ..ess, 1970--1973.
Nm'd F. }'. (ed.) Advances in Enzymology, Interscience. [Is8ued
anllually.]
.

75

Mosbach . (ed.). Immobilized enzymes. ': Methods in Enzymology, Vol. 44, Academic Press. 1976.

De Pierre J. W., Ernster L. Enzyme topology of intracellular


membranes, Annu. Rev. Biochem., 1977, 46, 201.
ll II


Per.~/lt .

Enzyme Stuctue and Mechanism, 2nd ed., Freeman,


1985.
Hirs . . W., imas('heff S. N. (eds.) Enzyme stucture. Pats
- . In: MethodsjJ1 Enzymology, Vol. 11, 1967; Vols 25
d 26, 1972; Vol. 27. 1973; Vol. 47, 1977; Vols. 48 and 49,
1978: Vol. 49. 1979. Academic Press.

McCormick D. .. -'ig/lt L. D. (eds.) Vitamins d coenzymes,


Parts A-F. l: Methods in Enzymology. Vol. 18, 1970;
Vols 18 and 18, 1971; Vol. 62, 1979; Vols 66 and 67,
1980. Academic Press.

7.m Nmlt',

1978. Recommendations of the Nomenclature Committee the International Union of Biochcmisty I the NomenclatUI"e and Classification of Enzymes. Academic Pess, 1979.

II
m .-.

(ed.). Methods of Enzymatic Analysis, 2nd


English ed. 4 vols, Academic Press, 1974.
. D., LardJ' ., Myrbiick . (eds.). Thc Enzymes. 3rd ed.
7 vols, Academic Press, 1970--1973.
l'ick S. ., Kaplan N. . (eds.). Methods in Enzymology, 69
vols, Academic Press, 1955-1987.
.ffi'nn-Slnhff . ' al. Affinity hmtg..h. Perga Press, 1978.
Jacoby W. . (ed.) Enzyme puification and related techniques.
': Methods in Enzymology, Vol. 22, Academic Pess,
1971.
Jacoby W. ., Wilchek . (eds.). Affinity techniques. In: Methods in Enzymology, Vol. 34, 1974; Vol. 46, 1977, Academic Press.

. . Aspartate aminotransferase, Test of the


Month 6, 1980, No. 2.
Bergstrom . Determination of serum alkaline phosphatase
tivity, Test of the Month 1, 1974, No. 22.
Cavanee W. . ' al. Expression of recessive alleles chromosl mechanisms in retinobIastomas, Natu..e. 1983, 305,
779.
Dozy . ., Forman . N., Abuelo D. N. Penatal diagnosis of
homozygous a-thalassemia, JAMA, 1979, 241, 1610.
Fishinger . F. Creatine phO!~phokinase and its izoenzymes, Test
of the Month 2, 1976, No. 6.
Gusella J. F. , al. DNA markers for nervous system diseases,
Science, 1984, 225, 1320.
. W., Chang J., Dozy . . Pages 275-283. ': Thalassemia: RecencAdvances in Detection and Treatment,
., Carcassi U., Rowley . (eds.), . R. Liss, 1982.
Kidd V. J. , al. a,-Antitrypsin deficiency detection direct
analysis of the mutation in the~gene, Nature, 1983, 304,
230.
Little . F. R. , al. Model for antenatal diagnosis of
thalassemia and othe m~nogenic disoders molecular
analysis linked DNA polymorphisms, Nature, 1980,
285, 144.
McNair R. D. Lactate dehydrogenase, Test of the Month 2,
1976. No. 3.
Orkin S. . ! al. Improved detection of the sickle mutation
DNA analysis: Application to prenatal diagnosis, N. Engl.
J. Med., 1982, 307, 32.
Pirastu . et al. Multiple mutations produce -thlssmi in
Sardinia, Science, 1984, 223, 929.
Southern . . Detection of specific sequences among DNA
fragments separated gel electrophoresis, J. Mol. Biol.,
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Wilk;nson J. . Clinical applications of isozymes, Clin. Chem.,
1970. 16, 733.
Wilkinson J. . Clinical significance of enzyme activity measurements, Clin. Chem.. 1970, 16, 882.
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gene allows prenatal diagnosis and carrier detection of classical phenylketonuria, Nature, 1983, 306, 151.


. .

5 11

Ix


,
,

-r ,

Y+R-X~Y-R+X.

u:

, ;

2)

1)

+ R - XpYRXpY - R +
'--Y--J

,
-

COCTOJIJI,

, ,

,-

I!G F

I!G D

, .
, (

(~,

).

opaHO~

+ RT In

[YRX]
,
[Y][R-X]

I!G o = A~ + RT In [ - R] [].
[ ... R ... ]

= I!G~

I!G F

)-, ,

I!G,

I!G

I1G F

+ AG D

. ,

I1G D

AG

I!G F

, ,

I1G.

.\f(!llm: 1\ullt'm""'a

I!G F

I!G o,

77

(I!G)

(. . ).
.

8.1

8.2;

. 8.3. l,
;l .
['" R'" ];1 [ ... R'" X]II; L\ (;:' L\ G~-u
1 UJ1.

I!G, 6GF I!Go .


. I1G . 8.1 (I!G < ).
. 8.2 (I!G> );

I!GF (I!GF > ), AGD


(I!G D < ). ,
, I!G
I!G F I!Go '

[Y"R .. X]b, ,
,
[Y"R"X]B.
['" R.. ]
,

['"

R ... ]_

, ,

I!G p , I!G
:


(.

Tor

I!CO = - RT ln Keq.

8.3).



.
, -

, yr
JJR .


, .

. 1.

r r, ..

, .
G<

2.

(..

),

r.
.
,
.

8.1.

J1 ,


. (L\ G < ).

.
,
. , ,

... R\ .

<

- +

. .


, ,

,
.

.
.

8.2.

II .


" (6 G> ).

8.4.

, -, -

,
.

78

(. .

),


(. .

8.4.

1: 1).

--.....,.~

---

, ,

00

. , (,

-6-

l-)
'

, .

'

S - y Enz

"-.Enz-S

. ,

, , 1

(. .

nz

. , (. .

AG < ,

),

D:G~E~~:G

, .

Enz-G

\.

; ,
,

. .
, ,

7:
D- G

+ A~A -

+ ,

D-

D-

, -,

, :

D-GZ
D

~ A-G

Enz-G..P.-

1.

, _ .

2.

3.

- S J'I

D - d .

Enz-G

Z-GJ

Enz - , Enz - G * Enz - ** --


Enz - S,

u .
,
( ) .

. . .

.
. :J

.
,

.
:

+:t

R-

(Kd )

: llmu

79

R - +=t R

+ .

[R][C]
Kd = - - - [R -

R-

8.5.

Kd

7 - S cor
~( .}.

,

.

( )
,

(Kd ). ,

(
) ~ .

. ,

,

, .

(. . 10).
.

+-+-

. ,

,
()
. ,

,

. ,

(.

8.5),

,
~
(.

8.6),

, ,

. ,

-
, .

, ,

, 1.


, .

.

,'
,

.
1

u. , ,
.

8.6.

() (S. S:;:) 'J


. ,
, rb (S.);
ra -

S2'

80

. 8.7.

.

( ).

,
.

.
, .

8.7,


(, ).

()

S- . ,

, ,

Lys

Met.

. 8.9.

.

(), ().
(),
().
-

~OM (1) (F.).

.
octatkob-Ls
(.

Met

8.7).

(.

8.8).

() ( )
.

(. 8.8,) ,

(.

8.8,)-

.
.
,

, .
, .

::e:::--:rtD

~
~

Q
,

.
. .

8.9.
,

.
,

.
,

,
() .

(.

6)

(.

9).


,
,

Enz + S

8.8.

, .

Enz S

~-l ,4-

(. .

13

33),

() (, ).

. ,

: ll

81

(, 101

21

Trp 62 '.8 Ser 100


Trpl3 Ile 98

Ala 107

Asn 59
108
Asn 46
D
rp
Asp 52[ Glu 35

Ala 110

Ser36 \
F Arg 114
.

8.10.

, ,

. "
F.
, ,

. 8.11.
CTPYKTypHOI"O .
. (. . 5.7.)

II .

, JO ,

(. 15000)
, 129 .

. JO

, ,

(.

KOTOPYJO

JO ,

His 12

His 119.

8.11).

,
-
.

1663.

J. Biol. Chem. 1968: 243,

, ,

~ ,


8.10). ,

(.

;
Asp 52 Glu 35. ,

8.1).

(.


. .

d

, , .

, JO
.,

st

Asp 52.

JO :

, , ,
.

. .

, -

82

8.1.

~t

(S)
3). (
Sequencc and Structure. Vol. 5. National Biomedical Research Foundation. 1972.

() . (.

Dayhoff . .. ed.: Atlas of roti

.)

r ()

(S)

VVSAA(H)CYKSG

DSCQDG(S)GGPVVCSGK
SSCMGD(S)GGPLVCKKN
SSCMGD(S)GGPLVCQKN
DACEGD(S)GGPFVMKSP

. ~

1.

( )GGV

( 11 )CG

VL AA(H)CLL



100 (QIO = 2) ,

10-

100.
Q.o.

2.

~l .

(.

QIO' .

.

8.12.

Enz-

+ +

Enz-

+ SH+ -

Enz - SH.

(Enz-)
(SH +):

Enz-:

- EnzH.

SH+ -S

+ +.


SH + Enz-,

~eHTa ,

( ).

Enz-

SH+

(.

8.13).

Enz,

S,

""

8lt8

.
,


, .

,

5,0 9,0.


O~------------------~

. .

70

8.12.

'\
.

83

: u

~Enz-

+ 2B-AB~

[][][],

[][]2.
,

+ -

n ,

PIIC.

8.13.

pll

[],,[].

~.

lI ,
( ,

;
(.

6).

, , ,

n,"


[., ].

ll ,

+ ~ n .

<t "",.

, .

k,

+ -.

+ ~AnB

CTb 1) (


. ,

1 =

.
1.

1)

k,[A]"[B]"'.

=k

,[n n,].

,
, :

1 =

_l'

k,[A]"[B]m = k 1[","].

[ ],

[][].

CTporo ,

, .

k,

k _1

Keq ~, -

84

1.

k,/k_ ,.

2.


. , . .

( ) .

. .

(1:).

II Il

IEnzl

(.

7).

l\J jJ ,

, -

" m .

"m

(' ,

4.

Enz - S,

Enz

+S

~ Enz

+ .

k_ 1

.
[Enz]:

(~GO)

CKOPOCTb 1 =

fiG

~G~

R-

= - RT ln

_1 =

~'

. -

k,[Enz][S].
k _,[Enz][P],

A-G .

= ~ = [Enz] []
eq

[Enz]

[]

[Enz] [S] = [S]

k- l

,
.

. . . .

, -

( ).

6GO).

. . .

, ()

K cq

6G

, .
.

(6G ..-; . ).
,

I- ,

Jl

6GO.

A-G".

. .
. .

. .

[S]

v (.

'/Jn,.

(.

(.

7).

8.14).

uu

, l0

. ,

. ,

. . .

1000 .

ullu

ll.

--------=.;:;;11..,---

85

}V 2

[S], Enz
S,

- ,

m. /

------------}V ./2

, d

[Sj

8.1 ....

( Vmaxl2),

111II !(UlIl/.IILlIt\ ~ri.l


r~1 . IIlii II.

100

/,

"':LJIli.

1000

.
:

[Enz] = 0,1 j = 10-9 ,


[S] = 0,1 / = 10-3 ,

,
.

{" [S] (.
8.14). .

. .

[S]

106.

10 000

100

[S] , v
[S];

. ,
. 8.14, . 8.15.

, .

+ S Enz -

-ll

Enz - S)
, . ,

Enz

S (

IS)

Vmax[S]
+ [S]

---..:=~-=-

nz,

(. .

IS).

Enz - S),

, -

8.15.

[S]

. 8 () () .

. (). , , , .
.

8.14.

86

1. IS) ( . 8.14 8.15).


[S]
, .
, Vmax ,

, -

. , :

=+,
=

l/v

l/[S).

(..
(..

[S]
.
+ [S]

1/[S),

l/v)

I/Vmax '

KM/Vmax ' , (
), ,
.

(~ ).

.'1 ,

=--=--.

J1 Jl0 (. . '
),

2. ISI
8.15).

[S].

( .

8.14

, . .


(.

8.16).

, ,
.

V [S]
v= max
.
+ [S]'

, r

IS),

8.15).

Vmax'
3.
151 = "'I
( .

{' =

8.14

Vmax [S]
Vmax [S]

v=
=
=
2 .
[S] + [S]
2 [S]

Vmax [S]
.
+ [S]'

, ,
,

[S]

. ,

. v
,

IEnz.).

,
"

, ,


. ,

[S].

Vmax

, )

[S]

[S),

Vmax

,
, . .

+ [S]

-.,-

[S] ,

=---

1
-=_._+
m

[S]

[S]
.
m [S]

, ,

-='-+-.

l'

1
[S]

max

1
[5]

. 8.16. -
( Ijvx I/[S),
V '

87

Vj

-=

[S]

v/[S]

V~ax

beh-I/

Vmax '

VmaJKM ,

Vmax

100

[ S],

V =

[S]

, r

Vmllx '

k- 1

= ,....

. k_1
[S] =--~ K d

= k2 :

Vmax
2'

(VmaJ

~- . -

.
,

+ .

-2

- -

Enz

( ) V ( ). ,

,-

Enz-S

-V-+--.

Vmax ,

k1

I/

= I/Kd
k2 + k_ 1 k l' l/KM

'*

Kd -
-

, (. .

--

Kd

Enz--S:

+S

10),

. fJ

[S]
8.17).

(.

Enz-S,

(.

k_ 1

Kd

Enz

6).

k_ 1

=-,;:.

Kd


. .

, ,

Enz

+S

~ Enz-S
k
-1

O~~

. ,

Enz - S

Enz + S

PIIC.

8.11.

_________________________

[S)

l" .

88

:. IVI

, (

).


(.

6).

02

,
,

log

t'

VmaJl

t'

- , (

) (
)
.

= n log [S] -Iog k',

. .

k' -

, [S] k',
n- [S). . 8.18
,

,
( ), .-
'r (

).

log (v/Vma.. - v)

log [S]

n, n-

= 1

n> I

n.

n < 1

(v
V/2), v/( Vma ..-v)
1 log [v/( Vma..- v) . ,
Sso ( ,

),

log [t,/( Vmax-V) =



(l) .

(1),

(S).

, Enz - S
. . - .

Enz - 1,

, ,

(1).
(S).

log

(. 8.19). ( 00C~H2~00 )
,
1
1

1 =n
__ -.J1

Enz - 1.

Enz --:- 1

"
"

-1

Enz-I ~ Enz

+1

-1

-4

Sso

-3

log [S)
. 8.18.
.
.1, , .

. = [Enz] [1] = ~
I
[Enz-I] k_ 1 '

89

.llm:

--
I
-2
-- -
-:;:::=====~.~

- --

11

- - -

"'1

-.

(v)

8.19.

8.20

R .

, .

Enz

S (1/IS)

= ) v ,

~ Enzl (- ~ Enz +

. ,

~ zS(.)'" Enz +

.)

. , 1/ Vm~x;

( ).

Enz-S.

(:-- 1/' < -1/ )'

, )'

(' ) .
,
,

(, ).

(Enz),

Enz-S,

Enz

+ ,

S,

. , (
) .

(; )
.

S.
Enz - S
Enz-I.
[Enz-S)/[Enz-I]
.

S Enz - 1
.
,

1 (. 8.20).

Kj

1.

[1]

.. ,

( ) ,

.
,
.

.
,

,
, .

S 1 .
S ,

.
,

IS
. 8.20. -
(. .
nOIIoe [S) ( Oj[S).

Vmax)' ,
1 S

Enz-I, Enz-IS.
Enz - IS

, ,

Enz-S;

. , -

l:.8

90

pEnzl~
Enz~

~ zlS-+Enz+

EnzS

Enz +
.

8.2]

]/v ]j[S]

(
,

lI).

(Ag+, Hg +),
2

. .

.
, ,

.
ll .

, (

).

, ,

. , , -

:
,
, , ,
, . ,


, .
aKT~HOCT

(. .

6).

,
, ,

10

[s]
. .2). )-) .'1

n HCK~lKYPCH fHnro Rtl.

Christensen . N. Dissociation, Enzyrne Kinetics, Bioenergetics,


Saunders, 1975.
Eng/e . . Enzyme Kinetics, Wiley. 1977.
Piszki'icz D. Kinetics of Chernical and Enzyrne-Catalyzed Reaetions, Oxford Univ. Press, 1977.
Segel 1. . Enzyrne Kinetics. Wiley, 1975.
Sigman D .. S., Mooser G. Chernieal studies of ~rn active sites, . Rev. Biochern., 1975, 44, 889.
Van Ta"'!len .. (ed.) Bioorganie Chernistry, Vol. 1, Enzyrne
AetlOn, 1977; Vol. 2, Maero- and Multirnoleeular Systerns,
1977; Vol. 3, Substrate Behavior. 1978, Academic Press.


.
, n

.
.

, .

?
.
.


,
(

n-,

(.

9.1)

n-

. n-
n-.

n--.

, ),
.

, ,

,

. ,

n-

) .


.
.

(, Tyr Trp)
R

(Met).

,
-

..

9.1.

n-113.

lOt

92

HF

I
~
I
Enz-CH 2 0H + F-=~z- --=
I
2

'

.~s

"(

s3

.~

C:D

9.4.

r:t Scr 195 , v 1


:r ().

. 9.1. n--
I1-ilu :\-.
.
n-.'Ia :\l .

n--.
-

-.


n-

-,

n-

-. n-

(.

, .

9.2): 1)

-.:

2)

n-

n-

n-

-.

Ser 195

-,

-S

n-

JI- ,
le ,

9.3.


- (-).

n-

- -

(.
(.

9.3). n--
9.2)

9.4).

Ser 195

.
.

__

27

() (.

-t.~-~ -
/

195.
Ser 195

. 9.3. 'I r !\10

Il-.
Jl;
- n-;

n-.'1 :
- -,;

n-
-: ' - l\l-. .IO
-' XT-Arl
-.

Asp 102, His 57 Ser 195.



, ,

,
.

His 57 .... Ser 195.

Asp 102 ...

: llU. u.

11
_
~
-- H-N~ N H-O-Ser
Asp
"---'"
I~
Hls
- (. 8. -)

1.

93

(,
). ,

4"-..
-C-O-HN N-H
11

bd

O-Ser

),

( -

, ),
. 9.5.
II Ser 195
(-).

Ser 195-

. - (
n-)

Ser 195

R-


(.

Ser 195

His 57

Asp 102

9.5).

-Srl95
.
, ,

55),

( -

).

? . .
,
(, ,


) .

, ,
,
.
, ,

lI

245

). (,
,

Ix

(. .

(,

~I

. , -

-,

1t-

13
_1415f16
_+--_ _ _ _ _ _146
--=--_ _149
---=-_____~24~5

(-), r

(.

9.6).

- , (.

9.6)

(.

16415 16
_ _ _ _ _ _146{
_+-+----'---_
_ _ _ _ 245
149

9.7).

,,-

14-15+ 147-14.!

-,
. ,

,
. -

13

;-,

146

s--s

s-s
I

~
I
S------~--------------S

149_ _ _ _ _-=-24:=-5
..:..:..:::

. 9.6. (-)
-IfIl (-) 11 . Jl
u-!\. (-).

S-S

16

S-------S

. 9.7. - ) u- (-).

94

, .
. .

, (

. ),

(
)

(
, ),

. m Jl ,

;

Cl ,

.

. .

1.

.

.

,

(.

9.8).

~,
'

(.

9.9

9.10).

,

,

2.

(
). ;
- .

3.

( )

4.

.
,

5.


.

,

His 57

Asp 102

~ - -,

Cl
,

Ser 195 -

- (.

()

9.6).

, .

, ,

~--4-~::QL

. ,

. 9.8.
v \1f1
Q .

9.9.

CX~\1a Jl-
.

95
AI.

E-CHO~(

--""".

'AI.

/ 2 NH 2

",

KG

~/

NH
1

"

.. E-CH 1 NH 1

Glu

J -

---+"

KG

Glu

. 9.10. I1- i. - - 2 NH:z- !\!.1 <1 .1 : . (Ala- ,


. KG--_1. Glu _"'IYTa '\1<1 .)

SH +

, .

- :
( )
( ).

d[P]

= - - = k [SH +].
dt

+ )+,

, ,


. ,
(
) ( ).

, 1 - .

[SH +] -

,-

1) ,
2)

, .

,
(
(

> 7)
< 7).

> 7)

< 7).

. :

. '

SH +

d[P]
dl

u,

k-

= k' [S] [ 0+].


3

[S]

[)+].

. -

(.. ~

7),

S+

+ -+

SH + +


. ,


~
.

S + )+ +t SH+

+ 2.

[ +],

+t

25%

. u

+ )+.

SH+ +

, ()

SH + -

().

; -

96

, ,

31

(M-Enz-S)


.
.

-,

),

( ).

, ()

/~

'.

. ,
, ,


. ,

,
.
. ,
, 8 ,
-, .

, 2

,
:

() ,

(Enz),
(S) 1: 1: 1,


()

,
:

Enz-S-M

+ Enz ~
Enz-ATP-M(H 20)2).

-( 2 )2)

M-Enz-S

,
,

Enz-M-S

, .'I,

Enz - S -

z--Sz",

I
S

Enz-

). .

( ) (:

1.
2.

Enz --.

, , ,

; . .

6).

Enz-

. aI,

.III": ..UJ ();um(IU.

97

JIl1

.' .t 1 '

Enz-M,

.
:
Jw'

9.1.

. .:1 1'0.11. ",

.,

, ,

(Enz*):

D-

!'

Enz-M + S;::::

z--Sz

456 in: fnzymes,


demic Press. 1970,

Mildvan

oyer . .,

S.: Metals in zm catalysis. Vol. 2,


Lardy ., Myrblick . (editors).


()

"- S

: 1)
- ; 2) ~
; 3) ; 4)

, "
. ,
,

.
,

(.

Mg2+,

9.1).

+ ,


(, +).

, .

()
-
.
,

(> 1)

-. ( ),


- -,
(

- ).
, ,

.. 4 1573

Crane F. Hydroquinone dehydrogenases, Annu. Rev. Biochem .


1977, 46, 439.
Fersht . Enzyme Structure and Mechanism, 2nd ed., Frecman,
1985. [ l- : .

.- .:

1980.]
Krat J. Serine proteases: Structure and mechanism of catalysis.
. Rev. Biochem., 1977, 46, 331.
Mildvan . S. Mechanism of enzyme action, . Rev. Biochem., 1974, 43, 357.
Purich D. L. (ed.) Enzyme kinetics and mechanisms. Parts
and . In: Methods in Enzymology, Yol. 63, 1979; Yol.
64, 1980, dmi Press.
Wimmer . J.. Rose 1. . Mechanisms of enzyme-catalyzed
group transfer reactions. . Rev. Biochem . 1978. 47,
1031.
Wood . G., Barden R. . Biotin enzymes. . Rev. Biochem., 1977, 46, 385.

10


, .

.
,

:
,

(
).

, ( 35 )
II -450,

,
.
, -

, , ,
II, ,
.

,
. ,
,


(cyr ).

, ,
,

P-4S0.

. :

XIX

'

, -,
,

,
,

. .

: zu

99

,
,

1.


,
.


.
,
.


. ,

10.1.

~ )[
.

. -
,

(. 10.1).

2.

.

. ,

.
.

,
.

OKPY~

. ,
, , ,

bIX ~

, , .

(,

),

(', ).
,

, :

1)
2)

);

3)

.
.

11 G

11 t1.


(k )

(k p8cn )

(.

10.2).

1 , ,

( k),


, .

. -

4*

( kpacn),

IlII

100

JO
.

~-,
~-, ' .
.
()

10.2.

(~-).

~ . .'

,
,

8 ..

k , k u '
.

k kpu.
( )
()
,

. ,
,
, .
, ,

).

,
, ,

()
()


. . . (. . 38

lI.
,

. -

40).

.
.

, ,
. -

() .

, .



(.

. , ).

(. .

, O'F'

. ,

,

. .
:

.

'.

,
,

41).

--- ,

G-

CoA- -450.

Escllerichia co/i,


,
,

~-lC, , -

. , -

ll:

101

aKmueHoCmfl

. ,
.

'l

. ,

Sa/mone/la (murium, (

Enz,

) ;

-4>~

E~

.
.
. :
,
, .

-l1llo

;/1

EnzL /

11

L..JI--r----..........
,-~

"

'"

EnzT


,
.

LL



.
. , ,

().
.

.
, ,

;

41.

. 10.3. .
Z L Z ,
.
.

.
.


, l
. ,
,
,

. ,

(Enz.)

. .

(.

10.3).

, ,
. , ~

(Enz L ).


, lp

(EnzT ).

Enz.

Enz2

20/0


, .

7-1Q%

(. 10.3)
, .

.
, ,

Enzl

Enz2

102

, .

CTd ,

( kll ),

Trp

( k.: ).

1 SN-

(.

10.1),

-, k

klt
,

4-5

. .
,

.
,
.

.
,

, -

.

1C0

. .;I

().

kr , k

, (.

8).

,
(. .

55).

k ~ .

, :

k ,

k:1 k '
.

, -

(. .

30).

103

10.1. , "

'.2.

100--120

+2
-2
+100
+20

20
60

15


-6- -

+ 10

D--

100

+10

+10

+30

-I,6-
ll /

'

-10
+30

f" ,

2-3

HMG--

5%

-10

10

mUJW n nllu

1%-
1%-

60
12

1, , HMG--, u.

, .

in vitro,

Rev. Biochem. 1970: 39: 929.

lC

-10
+2
+3

in vivo .
, in vitro
, in vi-

vo.

.
, ,
\


.

, ,

.
.

(
)
,

17.

.tlf 10

j ~

1.

, ,

. ,

, ,

. . "t

d ~.
,

Mg2+ Mn2+

, . ,

Mg2+,

Mn2 +.

in vivo.


Il

ATPjMg2 +,

, ,
. ,

.
. 2,-

. .

Enz(

-+

Enz., ...

Enz z

-+

nz :
.,)

~ ,

,
1l0 .
-

.
,
, ,

Enz .

. .

,
.

, .

R. .

, .

in vivo

, . . 9),
, ,
. , -

Enz. D .
D

,
.


rn ,
,

.l('um: mllllm

105

, n .

(,

5 , - 5 2 - 5-5.~

~c

).


1 ,

110

.
- ,
- .

. 10.4.

S.- Ss --

His, Trp

. .

10.4

,
(

). S., S2 S
(. ,
), S4- ,

ss-

S~.

-.
,
.
.
-

ll

.


,

.

i
,

" ,
.

S4~B.

S4~C,

e (01),

S~Ss~D

. .

coli
(AK L )

, (AKt)-,

10.5).

, .

S2'

(.

(AKh)-,

Met, Thr
AK L ,

(.

10.6).

Lys

ll

. -

,
,

. , ~
.

Te:IbHO , ,

, ,
. ,
,

,
.


Q . I

. (

) .
. . ,

!!

G.

. 10.5.

.
( ) (
), ,
.

.u /

106

10.2.

t3cn

coli ( 1)
col; ( 11)
. coli ( 111)
R. rubrum
. subtilis

11L..._ ___~__ _......

/"

Lys

Lys

Thr
Thr
Thr + Lys

() .

.
,

. 10.6. ()
E-coli.

,
.

(,). () (),

,
.

.
.
,
.

1963

, .


.
11
--

. , .

, -

L-

10.2).

.
-,

. '
(. 10.7). -
()
().

,
. .
.

11

--

H2 N

"" I

........ N"

"""
........

I,..H

+
+

/,
/11
2'
N
-

. (.

........

H2 N

. u

10.7.

( ).

107

, , ,

( . l.
),

: .
.

. ,


(. 6).
.

. .

. .

. ,

1.

, .

. . .

R (

) ,

0--5'-' ,

2.

[S]

. .

, .

. .

[S]

. .

, .
.

BToporo

. -

.
.
.
.

00

4.

Q.

5.

(. )

lC
.
.

10.8

. u

.

O~~

______________________________

[S]

. 10.8.
r .

108

. 6
.

JO
,

:
.

, .

02

-
V-.

( ),
V .

V"

V-


; r

in vi\o.

( ),

V' ,

. ( ,

V-

II -

, Vm..".

Vma".

,-

- ,

. , ,

, .

CoA-);

(HMG-

G-- r.

,
HMG--,
.

02


. , ,
-

~M'2+~

C!nz-SVP-Enz

.~

~O-PO~-

:>-

OH

I'IIMP-Enz

Mg 2+

. 10.9.

f_
- d.
-- . u (NTP) . .

Pj

. 10.10. .

/ .

(
) (

J'I ). ,

( -

, -)

10.3.

,
(.

Icl.

10.9).


}. ,

, -

-. (.

10.3).



-;

--

HMG--

HMG--


-.

(1-3)

, ,

; , ,

, .

6-

-6-

1,6- (. .

18).

10.10,

( )

()


(.

10.1 ).


(.

10.3). ,


().
l
.

,
.

( ll 1
3) (~ 2 4)

1.
2.

+ ~ ADP + -6-
+

-6- ~ ;

ADP + ;
3. Enz-Ser-OH + ~ ADP + Enz-Ser-O-P
4. 2 + Enz-Ser-O-P ~ ; + Enz-Ser-OH
2

110
li




.

;
.

, ,
.



. ,

10

Gumaa . ., n ., Greenbaum . L. Compartmentation


in relation ( metabolic control in liver, Essays Biochem.,
1971, 7, 39.
., Grisolia S. Biochemical Regulatory Mechanisms in
karyotic Cells, Wiley. 1972.
Nestler . J., Greengard . Protein phosphorylation in (
brain, Nature, 1983, 305, 583.
N'shlm . ., Stuart . Regulation in Met~bolism, Wiley,
1973.
Schike R. ., Do)'le D. Control of enzyme levels in animal tissues, . Rev. Biochem . 1970. 39, 929.
Soderling . R. Role of hormones and protein phosphorylation
in metabolic regulation, Fed. Proc., 1982, 41, 2615.
Sols ., Marco R. Concentrations ofmetabolites and binding sites: Implications in metabolic regulation. Cr. . .
Regul., 1970, 2, 227.
Stanbury J. . ' al. (eds.) Metabolic Basis of Inhcrited Disease, 5th ed., McGraw-iIl, 1983.
Umbarger . . Amino acid biosynthesis and its regulation.
. Rev. Biochem, 1978. 47. 533.
Weber G. (ed.) Advances in Enzyme Regulation, Vols. 1-9,
Pergamon Press, 1963-1987.

11

11

,
,

. .

R.

. ,

.,.

,


.

. ,
,

. ,

( G)
( S)

AG

(, ). ,

= /). -

, .

TAS,

(),

/). G = /). -

S.

G ,

; ,

. -

( G) -

112

//

~---------------}
~+

. ~ G
,

~~

/). G ,
;

.
.

/). G

G
.

1,0 /
/). GO-

.,..

~5J

1!b1::

.q,.

....~

1'1

'7~

cv

CI

ID

7,0.

II.

11.1.

, ' eq:

/). GO'

- . + D +

/). G()I .

R-

2,303 RT log

= -

, -

(. .

',

. G

84).

(]'
.

()If ') l
LlIl.

/ ,

.
( -

)
,

.
.
, .

11.1,


-:- ,

1,

+ -+ 1 -+ + .

, -

. ,

11.1.

, (

) ,

( ri

),
(.

11.2).

, .

. -

, _,~ ,

~"''2

113

11.4.

( '"

0)

().

PIIc. 11.2. r r
r l.

(.

11.3).
. 11.3 '" ()

() -

. G)

. .

'"

()

D.

Ali


.

, :
;


Fe2 + -+ FeJ +.

, .


(.

11.3

11.4).

. 11.5, - ,
. . ,

11:
S

'-

Q.

1'1

Mg2+ -

(.

11.6).

11:

Z
CI:

10

(D) (;).

11.3.

lCr
"(.

, ,

NH 2

2~ ?/........

(XN>

1 ~

80t

0-

IC

eCJC

11

1/0,

A'~

0-

-0-~-0-~-0-p-o-eH2
11

PIIC. 11.4.

0-

er lCr .

11.5.

().

([([

114

,
,

M g 2+

\
\

0I
- 0- -

0-

0-

I
-

I
-

11

11.6.

JJ

11

( , -),
, ,

, S- ( )

11

UDPGlc

().

MarHllcBbIli (,10Il ~f
~4 \1 ~'1 g- [) ).

,... 0,

() .


. 11.1.

/). GOI

37;) ).
/). G'
, - 30.5 /.
. -
,

'"

, ,
,

. B-
, -

11.7).

(.

;
, Ci
.-

/). GOf

. -

l!GCf

~He

. .

D,- (,
1 1.3-),
(, )

(,
).
.

J ;t,l

11.1.

'

1.3-
(:

-61.9
-51,4
-49,3

+ ;

-I-

-6-

-6-
-3-

"

l:::.G


ll (
) ,

jJ . .

ATP/ADP . - 0.
u, - <V (. 11.8).
fJ'IaBHblX .-. 0,

-43,1
-30,5
-27,6
-27,6
-20,9
-15.9
-14,2
-13,8
-9,2

-10,3
-'7,3
-6,6
-6.6
-5.0
-3,8
-3,4
-3,3
-2.2

(.

0,

Ci

129j. 2.

l~.

<v

-'
(. ,
18.2) ,
(. 11.9). 3. J
. - 0
,

(. .

1.

. Ci r ,

....

Krebs. Komberg. 1957. [

,- .

,
Ci .

..

. .-- .: ,

1959.)


<lU (. 11.1)

JJ I'.

3-

-+.

.1ll.

-14,8
-12,3
-11.8

17.3).

, .


; ,
, -

Ullmu

0-

0-

0I

115

6- -

tI

+ ; -+ -6- + 2
(~GOI = + 13,8 /).

-- ~

(1)

-- -- -----

'{

"

()

0I

0I

".

11

-- ---

(2)

-0-

..... D

+ ;

(~GOI =

(1)

30,5

(2)

/).

.

:

()

, .

0-

I
0- --

+ JCJ('J -6- + D
(~G';/ = -

11

.
:

---

--

16,7

/).


>

()

11.7. .

l'

(). -

(-).

,
(.II.l).




.
,

1,3-

V"n,~ I/ ~:=~
.---- . . .~--

, ,

(.

11.1 ).

,

(.

11.1),

. :

-'l,1JC

+ -0 -- 0 ..... 0

-0

+ -0 - 0.

ATP/ADP

ov':: ~~-

8
.,.

-3-

-6-


(.

11.3).

(. .

11.1

18.2).

-16-

. 11.8. /
l' . }f,
l' ,

l'.

116

11

..

1'\

--

t
CH z --@

--

I
CH z --

1,-'

,-

11
CH z

11.9.

11

I
=
I

()

()

R1 ", ()"I Jl}'I' I Jlt"\JJ! "' ,

1\1

+ - ...,---+

-- 0 - 0

()

--2-0

'" 0.

-r!l11t

(2)

, II
(;);

Itllil!l

1)

2)

. ,
,

3)

'I!lr

()

Ari-0

+ CoASH + RCOOH---. + PP +
j

+ RCO - SCoA.

. ~

PP j ,

()

(.

217).

8
8

-N

r '

'\.

-N/

C=NH

11.10.

H2 N

=- -N
~

(~GO = - 12,6 /)

"
N
/C:8 H

~
.

8 . .


d.

PIIC. 11.11. .10 \1


II,10.

117

UJ1Imu{/

/).

.....

/). G':>1 (- 4,6

0.

Pj :
ll

Il

PP i +

lJukll

.. 2Pi

,lJt

+ - 4

+ -0 '" 0.

11.11.


u. ,

, I\ ,
:

i!l"lla

+ UDP ...----> + UTP


4

()

+ aOP~(----,AOP + GTP
()

+ CDP ...( - -

ErnSler L. (ed.) Bioenergetics. Elsevier. 1984.


Harold F. . The Vital Force: Study of Bioenergetics, Free,

Press. 1986.
. .. Kornherg . L. Energy Transformations in Living
Matter. Springer, 1957.
Lehninger . L. Bioenergetics: The Molecular Basis of Biologil Energy Transformations, 2nd ed., Benjamin. 1971.

Krebs
.+
()

]986.

Klotz 1. /. Introduction to Biomolecular Energetics. Academic

12

- -:

-
,

- ()

Fe2 +

. ,


. ,

().

() ~


. .

GO'

._

JI

12.1.

- 0,42

--

. ,

, . . )

FeJ-t- /Fe2 +
Fe 3-t- /Fe 2+

: ./.
: Fe 3 + /Fe 2 +
/

i (<< ): ./
/

-450.
,

: ./.

.
, .

:.

+0,82
+0.29
+0.22
+0,10
+0.08
+0.03
-0,12

d ,

; ,

(');

7,0

0,0

()

11),

(. .

/J3-
NAD~/NADH

H+/Hz
/-r

-0,17
-0.19-0,27
-0,29
-0,32
-0,42
-0,67

,lO

119


, .

12.1.

AH

tryrc

Y202

(OCC.H~K"'YI".1 ,

, ,

HzO

(.)

.
.

12.1.

,
.

2 2

2 2

1)

..

AH2~
~ 2 2
(

l.

( ,
.

1 2 ) (.

12.1).

z
AH

(.)
(.) (Bocc~aH.)

- ,
;

' 2

12.2. . 1

2)

, , .

, (.

3)

Hz

(.)

12.2).

-, -,

(.)

(.)

. 12.3. ."I.
( ).

. .
.

(.

12.3).

.
. -

;:
,
.

. ,

(.

12.4).

I I

I I

2h~~~ H x Z
~ 2

(.)

(.)
.

(.)

Hz

(.)

(Boccraj

(.)

12.4. .

(.)

H z

(.)
-

202

120
-

4)

12

CHl -
1

--

().

.
: ,

- ~ -

, , ,

--
I

--

, . ., 1,
.
-

5)

yY'NX:NI~O

~......::
N

NH
11

12.5.


, .

FMN

(.

12.6);

F AD

(.

12.7).

FMN F AD - ~

. ,

, ,

L- (

. ,

L-)-

L-.

();

, ,

Fe3+

Cu,

Fe2+

(, ,

) -

FMN-

( -) 0. ,

,
,
,

CHl

(F).

(FMN)
FMN FAD

(.
(

82)

HJC~~NH
N
11

, ,

I
--

'NNI~

12.?).

- -

H-~ -

er

--

().

-o-~-o-

12.6.

FMN).

(.

121

,/luII.(J lIlI

11

11

----- --0- 2

--

[). H-~ -
{ - -

NH 2

H-C~H\ [),~~N:""':::)N
H-!-OHV
"H-C--+----N

,
I

--

HJCyYNyNI~o

H1C~~NH
N
11

.,.

er -

12.7.

F D-

, .
,

N-

,
,

NAD+

(FAD).

.
,

.
- FD- ,

. ,

NAD+

(.

12.9).

(.

) (.

12.8).

12.10).

ND- er
-

. ,

, ,

. ND- er

(NAD +),
(NADP+),
(. 12.9). ,

;
.

, ,

, ,

NAD+, NADP+.
NAD+ NADP+

---

() -

. ,

.
. NAD+ NADP+

<;

12.8.

.,

122

12

~r----- - - - -....~

~-N2Il

~N

11

--

PAPPi-;

t+-

- o~
-

c-o ...
-........._ _ _~~...\~_ __

11

11

(ltl ~,":~,

1/

,
PPj

PRPP

11

--

11

(NMN)

1-:,

NH,

N~N,

o~-o- L...NJ..N/
2 , I
AH~o,

o;y~

0,-/

i.

0
?'

-ND+

OI
.

ft

C-NH 2

11

2--3-60-

vanr:

(C
~

~-O-

NH2

N:):I

N'1
3-:
.
/ ,..,....

'N

~H2
}

ll

--

.... ......... NH 2

NHl

0= rfI

0,-/

O----r.

'~

(. . 31.19)

{ ciR~
~38(XjCH2--t
~ 0,_/0


....
I
0,-/
.

-.

NAD+

I
NH,

. 12.9. (NAD). 2' - (


) (NADP+).
, .

. io .
5--I-.

PR -

123

II.IO.,u lfu

Q-CONH'

"

~CONH,

++

~~)
N
I

OCONH'

,
"

N
~

R
NAD+ +

2 .....
__---tl.~

NADH + + +

. 12.10. .
( 2 ) 4 .

(-, -); -,

;J' . , , .

. .
,
,

, ,

. ~

F .

FMN

. ,

NADH-

, .' (:,
().

NADH

-,

-
-3-

Fe2 +

Fe3+

( -450
.

bs)'

) (: .

18.5).

NAD+

(F ).

on :'

CoA- (. .

13.3).
. .
,
.
-

. .

, ,

(. .

333).

. ,
,

, , ,
. ,

, ;
:

2 2 + 2 ....

2 +

124

12
:

,
2 2

. .

2 2 ,

vivo

, . . .

ln

2 2

2 ..... 2 2

3-s-
u
, ,

L- ()
.

. (

, ).

.
; "

+ 02

"
+Z

():

+ 02 + ZH 2

--

+ 2

, .
, .
,

-450-

, ,
, ,

-.


, ,,.

. (. 12.11).
, 2 2

.
Il -450

NADH

NADPH

(.

12.12);

bs


,
(.

12.1 ):

+ 02 + 2Fe 2 + + 2+ " .....


(-450)

. ;

-+ -

+ 2 + 2Fe 3 +

(-450)

.
:

1)

2)

( -

).

, , ,

. (-,

. ,

).

-450.

02

~===~=~l====...
R

' 2

'

~
H'l02

I 2 2

:\',~
f
'\
' 2 2

02

.iJ

-450-

, ,

;
(
22 2

11

18).


2S-

l)

125

t.re~ 1 CN-

NADH . .

ND-.,

---..

apn-<:-

-450--'

" '. .- - - - - . . -n
"

-""

-Q
.

12.12.

(CN -)

. .

1 24;
26
.

. , -,

- (;

), ,

-450 ,
.

( ),

:
ND- FD-
,
Fe 2 S 2 - ()
-450 (. 12.14).

- .

~ -

2 2

Enz-H 2

+ 0 2 - - - - + Enz-H + O~ + +.

----------..-450--

1+ ~
Fe
-450

NADPH-

P-450-88

-45--

F +

Fe2+

-450--

Fe2 +-0

-450--

Fe 2+-0-r'
2

A-QH
. 12.13. -450- . I"
. -450-
Fe2S2 () ~ .

126

/2

, 2 +
Zn2 +; ,
, .
Mn2 +

IOO%-

.
12.14. -450-
. Fe 2 S 1 -- (
). Il NADP(H)
,
,
, J1 ND- .

( ).

, , O.~,
.

02

+ (F 3 +)

.02

+ (F

+).

-
:

I il

.
, -~, -450,

u (.

12.13).

Bonnetl R. Oxygen activation and tetrapyrroles, Essays Biochem . 1981, 17. 1.


Ernster L. (ed.) Bioenergetics, EIsevier, 1984.
Fleisher S .. Packer L. (ed.) Biological oxidations, microsomal.
cytochrome -450, and other hemoprotein systems. In:
Methods in Enzymology. Vol. 52, Biomembranes, ,
Academic Press, 1978.
Friedm'ich 1. Superoxide dismutases. . Rev. Biochem.,
1975, 44. 147.
Salemme F. R. Structure and function of cytochromes , .
Rev. Biochem., 1977, 46. 299.
Scllenkman J. .. Jansson 1.. Robie-Suh . . The roles of
cytochrome bs in hepatic microsomes. Life Sci., 1976. 19,
611.
Tolberl N. . Metabolic pathways in pcroxisomes and glyoxysomes. . Rev. Biochem . 1981, 50, 133.
Tyler D. D., Sutton . . Respiratory enzyme systems in mitochondrial membranes. Page 33. In: Membrane Structure
and Function. Vol. 5. Bittar . . (ed.). Wiley, 1984.
JVhite R. . n . J. Oxygen activation cytochrome 450. . Rev. Biochem., 1980. 49. 315.

13

()
Ie

-
(

).

13.1.

13.2)

(.

12.1.

. (

, ) (,

) .

NAD+/NADH

02/20

1.1

ND- ,

ND-

, .

; ,

l-

u (, iu. .
. 12.1),


,
,

(.

13.3).


(- ~'I "J).

. ,

II

ll

(.

,
.

. -

13.4),

128

J3

"

/J-cn

n n

cn

cn

.enwt.

NAD-

n.80

r- ~

YrneOA

er-

.....

reH.3bI

re83bI

30n

8an
.n( an

. 13.1. . .

'

NADH,

NAD,

. . ,

-,

, .

); ,

F .

NAD

L ( + )---, D ( ,

)-3-

NAD .
NADH

NADH-.

FMN

, - ,

FeS

). (

FeS

) .

 .

, , -3-, ,

(.

13.5).

13.3.

- (.

FAD.

+ -+ +

13.2.

13.3).

:: NAD+Y:~~~~~T::~Y H,D

NADH
2Fe'
V,D,
+

(.

13.3).

2+

---t2+

--

mll

129

--
-

(FAD)
FeS

8----.-----..ND ~(FMN)---=a ~ .......... ,--. --. ....... 2

J'

(FAD)

a-8

Fes/+

FeS

Cu

()

(FAD)
FeS

FeS

t
t

(FAD)

~tl --

. 13.3. . FeS 02- .


; -nI.lUi ; FeS-)~ ; -; Q-.

..

- ++

..

( )

(Q);

~ ( 1 = ~CH2JnH

()

CH'O~CH'

13.4.


()

- ,

10,

..

f>-1fI.

()

,
-


,
,

02.

, .
r
.
. 13.5. - (Fe.S 4) -
. S - " ; Pr - ; Cys . - " :'
2 2 (Fe2S2).

5 1573

130

/3

anI

I ....---!I-~

11

FoD

FeS

H 2S

HfA

8AL

CN~

NADH .............---1.~IL..-_ _- - ;_ _---J~~. . . Q

I
I

oIan

Cu

02

Cu
I
I

I
I

IV

feS,

I
I
I

\
PR8

111

. 13.6. ar Jl () ,
. . Jl
. L-; F- . I-NADH:
; II-: -; III-:
.Li.; IV - : JI-. - ,
. 13.3.


. ('

; -
,
1 .

(.

, ND-

. .:l

, .

'l ADP.


.
))

13.6).

(.

3
3 1/2
. : = 3 (. 13.6).

2 . ..
: = 2.
lO

. 1

11.8).

61

- r (. .

18.1).

42%

, ,

. .

. . -

2780

huumlO vuunu

mltv


.
,

, . .

13.1.


, illl

1
2
3

B~ex

5 ,

(.

4
5

13.1).
,

131

4.

. ,

,
;
,

(.

13.7).

(, ,

5:

13.6.

. ~

J1.

, ,

/-
(.
138),
.

-, .

, ,

),

1.

(4{}-45%)

,
, ,

(,

NAO-

. ,

.-- u. .

13.7.

lC .

132

13

4l

2,4-

(--

).

100

8308

NAOH

NAOP.

), (

).

13.8.

: 4.
D .
D
4.
, , ,

.

D. .
, ' .


. ~]

,
. ,

l",-

(1 -- ).

. -1S,

TTFA

()

(
: Harper.
Rodwel1 and Mayes .. 1979).
,

Q.

(, +)

, .

(.

13.8).

. Q

r
.

13.16).

( +)

(.

),

. ,

, .

, -

133

utt Utttt

~~
(.1 Jl '),

( ) .

NADH

NADH
6

FMN

FMNH,. FMN

n~,

Ll ( ,
(.

.

.

FeS-,

TP-).
;

13.9).

FeS-
(Q). ,

. QH,.
QH:!,

(/) ,
1, 111 IV .

13.10.

0/8 ,
l .

13.11.

. ,
-

. (

b S66

s ) ,

, -

. -

2+

. 13.9. . , F. F-
. .
, ;
1, 111 IV. . . ~
. + ' . .
F o.

I1_ _ _.
..r.IO

I
_ _ _ _ _-+-_ _8__..;....PI_._ _...._ _ _ _ _ _ ~

,,

:):'

I
I

2+ ..._ _....,:::~~2

,::

%+2+

I
I
n

I
I
I

~11....t - - - - 2

2H+~

2---

~-~2e-

(/)

~2e-

:
.13.10. lI-

(l:

);

0"
., -.

8....
Il1.. ... .

(8, -.)

NAO+

2+

2+

+
+

"O"-n

13.11.

(lf

). 1
;
q,

Q It ,

. .
Q-.'I.
:r.

( ),
. Ifa .
ltllO Q , QH 2
Q .
, , .
( "

lI
).
FeS-
(J- .

-
(. . 13.9).

u('umI~~ uutt

135

~ ,

QH2

('.

Q-,

(.

() -

r . ,

/ . ~

, . 13.13.

QH 2

13.11),

a J,

j,

Pj , ,
. ,

( +)

, .

;
, , ,

, .

F .

(.

13.11.

:r

13.12).

1.

F.-;
- (.

; .

13.9).

2.

F.-

bI

-.

(. 13.9).
3.
, ,
,

(.

Fo,

, , (.

(Fo-

13.9).

F.)-

. .
,

. -

4.

13.11).

: + -

6plI nlt

ll1ltI" IfICI.

O~O"BW"'CA r
8'"

. 13.12. . ( ll
. , r1
.

136

13

pv

F.I

"-

2+ ~--~.-O-P ------

1-'" _ "1 "1

"I "I

+ O-P-D-P-O-A

\
0-

1 : 2,

13.13.

-1

4 6 ;

,
. uu

2.

. (

. )
(. .

164)

(.

1.

- ( ).

+ :

3-

"
11
"
-O-P-Q-P-O-P-Q-A

13.9),

-
.
.

x
(. ).

3.


,
,

.

-
+.

.

.

(),

( )
(.

13.12).

(. --,
-,

-. 2 ).

.
.

J3-0
;

(
/ ). 3-

-3-

,
l
.

um U.fuu u

..

--:

ND+~,--

FAD

__
-_ I
.
L__.=:.,_.:..;:,-_3-

-~

rl

NAD
+ +

137

r8

FADH 2

..Il.... ..
_

13.14.

\ 'JIC3."I \1.

'" NADH

NADH

;
,
(. .

NADH

18.2).

.
.

. ~

.
~or ~
. 13.14. ,
, ,
0"

NAD+)S

I
NAO

.
,
, (

, )

-3--r .
,
,

.
8 .

13.15.


,
-

an

"

NAO+

M''''_~~'

an8rl

t<COJOUllf

8OI8

Il

NADH

+ +

Il

VI8

I-

.
F D-

+ +

NAD.

13.15. ~ - BHYTt:b .
lC, 2 -- .lC (
).

138

13

, ~

ADP,

ADP
13.17). Na +

(.

+ .

,
2+

+,

, ,

Na +,

2 +

Mg2+,

, 2+ / +

Na+.

ADP.

IItIA

N-

(.

13.16)


, . , , 2 N ,
, ,

3-

, .

2 4 --~~~~~~-.
N-
I
V8l-

----IPIIIII.....--=---1---t~

(. . 23.1);
,
,

, .



;
, , ,

2 "4

Ofl-.


. -

. ,


. ,

. 13.16. If
. 1--R . 2- .
3 - , 4 - If l
,
5 -
-.
6-IJI\: . N-.'1II,
UlI ll ()
. ( )
(. . 13.15), . lI, (. : 23.1)

uumlO l'uunu

an~

pl

139

mn"

.
. +

.
+ , +;
2H+--~--~---------+----~

,
~.
, , ,
.

--4~+------. D

----ho--.c+------------1
----~~------~H+



~

.

13.17.

1 (1)

ll !1 (2) II.
. H i /P j m aHTIfJlOplY PJOII-I
lO 13.16: lI
l1.ffJf I
Il r. llO .... IQ

, l, . 31(\
1J1 rr(' : 3 II
.'1II

ll (8 ll 01 IIfrt , i1!r
TO.;lbKO ); OH.I l'lt
JI rI10~ (. 13.11) (2- \
ytJaC1 )

3li 01 /, 2 NADHl :l 1,3 .'1 lI IClI (


III\l).


, .

.

,
. -

mechaoism aod regulation of synthesis


F.-ses, Aoou. Rev. Bioehem., 1981,50, 681.
"

Cross R. L.

. . Rd,'ll

V. W . Mayes . . Page 276. 10: Review


of Physiological Chemistry, 17th ed., Laoge, 1979.
HateJi . mitoehondrial electron transport and oxidative
phosphorylation system, Annu. Rev. Bioche"tn . 1985, 54,
1015.
Hink/e . ., MeCarly R. . I-Iow cells make , Sci. m.
(), 1978, 138, 104.
Hink/e . ., . L. Th phosphorus/oxigen radio of mitochoodrial oxidative phosphorylation. J. Biol. m . 1979,
154, 2450.
Mitehell . KeiIio's respiratory chain coocept aod its chemiosmotic coosequeoces, Science. 1979, 106, 1148.
Nicholls D. G. Bioeoergetics: lntroduction to the Chemiosmotic , Academic Press, 1982.
/ D. D. mitoehoodrial syothase, Page 117. I:
Membraoe Structure and Fuoction, Vol. 5, Bittar . .
(ed.), Wiley, 1984.
/, D. D., Sullon . . Mitochondrial traosportiog systems,
Page 181. 10: Membraoe Structure and Functioo, Vol. 5,
Bittar . . (00.), Wiley, 1984.

14

( -)

.
1) '
1 .

. , ,.

.
;

- ,
, ,
, ,

( 4 4 )

ll

(6 ,2 6 )

2)

(CsHIOO s)

).

, .

3)

3--6

1.

4)
6

.
,


- ,

, ,

-.

ll
,

141

11

11

I-

- -

3 2

(~)

(L-)

) .

D8

L-8

(, .

14.1,

I
H()~C -

,
.


, (.

14.1,

I-

).

1
- 2-

- -

1.

- 3 -

(.

14.1.

11

11

- -

).

- -

- 4 -

HO-s -

II 2

[).

L-8

, .

14.2. D-

L- .l .

).

()

2".

. .

16

1. D

L:

D-

L-.
.

~---o

).

L-

14.2

D- .

D-

L-

- - ,

(
-

- -

5).

( .

14.2),

D-, ,

L-.

.
.

14.1.

-D-.

142

/4
I

J----Q

.
,

( .

+).

-). ( - ),
D( +), L( -) L( +);

D- L-

a-D-t<8

,
. ,

( - )-.

D-

L-

. (
DL-).

, ,

2.

a-[)"t<8

,
(.

14.3).

. . 14.4.

(,

D-

D-

; . 14.4).
99%

1% -

3. - -.
- .

(.

14.5),

1,

1%

(36%)

~-

(63%);

~.

:
,

-----

-- -

(:)

1.

,
(

) ,


0.0025%.
; -

( -

), ()

4.
2

1----0

. .

- -- .
. J

. 1
,

5.

a-[)..t<

14.3.

14.6).

--.

, ,

a-D1t<8

(.

2.

(.

14.3

14.4).

143

~---O

J...----

a-D-non8.. ' \ . .

13-D-r

14.5.

D- (

IQ

)
L- ( , .
14.9) L- (
, . .

21.1).

, ,
().

, ,
,

(.

14.1).


, , <) (. 14.2).

. 14.7. ,
, .

14.8.
.
,

)
,

( a~o
). ,

--)

HOCH~

}----:.-

J----O

}----

..

a-

a-

14.6.

-. -

bl.

-[)'

144

14.1.

J4

D-

(: , NAD, NADP,
).

D-

D-

D-

, -

D-

L-

,
,

. .

. ,

Bo~poa.

, - . ,

( ),

(.

14.11),

().

Na+ / +-

(.

14.2.

D-

14.17) -

14.10).

r :r

()

. .

D-

L-

2- -

.
, (.

; ,
-

D-

D-

ll 'u

145

--

2
~(~~)

I
--

--

- -

- -
I
2

t
I

I
- -

D-

D-
I

--

--

I
HO~C-H

--

I
--
I
- -
I
2

--
I

--

D-

--

- -

- -
I
- -

--

I
- -

--

- -

I
- -

- -
I

- -

I
- -

D-

--
I

- -

,
,

--

D-

14.7. ,

J]. D-. - Jl0 l R.l.


J 2- - eHO- :!.

2
2

I
=

=
I
--

=
I
2

--

- -

- -

, I

I
2

~
.

14.8.

=
I
--

I
--

I
- -

- -

- -

--

- -

I
I

I
I

[).

D-

( ).

146
-

J----O

~---o

14.9. -D-

J4

() J3-L-1 (

14.11.

2--D- (J3-).

()

J-----O

D-

(.

14.(2),

D- D--

.
.

()
(. .

54).

+ t1

(, )

. ,

. 14.12. (2--D-JC) (
).
- 2--D-JC.

. " N-

, , .

,
,

(.

14.13).

(.

14.3).

, <<

)):

.
Q- .
,
,

, (
)

""--0

, O~
H,CNH

HNCNHz

1.

NH

14.10.

!"f () ().

147

'

().a-D-r I- (1 - 4) .cz-.

().a-D-r - (1 -1) --.

....- - - 0

1-----0

J-----O

Joo----O

1----0

().-. n- (1 -

2) -13-[).

0-13-D-r - (1 - 4) -fj-o.r

1----0

J-----

13-

. 14.13. . -
aTo~e

14.3.

( ). J1
,
( ). .

(
). ,

.
Jl

. ,
,


,
Jl

148

/4

14.14. . - ; -- .
1-6.

~?H'
.
.

o~~

~----------------------------~

. fH2
1

. 14.15. . ---: . -
. , . R-
. , ' ;
1~4 1-6 !J 12 18

llllll?Ut'll .Il .l

149

, .
:1

(15-20%),

14.14), (80-85%),

(.

4)- [

(l -

24-30

6)-].

(1 -

(. 14.15)-,
.

HOCHz

, ,

11-18
u(l -

-- [

4)- ],

6)-

'

, .
,

I3- ~

-4-

( 6-)

.
,
.

.
--

13(1 - 4)-

I3-

I"+

.
, ,

, -.

.

'

NH.SO:J-

J3-,

14.16.

bl <tJR

. , ,

N -- -
, (1 - 4 )-
(. 14.16).

, .

()

,{

, -

150

14

h:_NH~O

~~g~

, .
f' ,

f' (.

14.16),

54.

()

(. .

42

54).

15

N- ,

( = --).

( ),
(

14.18.

),

.
,

()

(Ga1)

. 15 42. ,

f'

N-

N-

(GlcNAc)

(GalNAc)

, ,

5%

. -

(Ara)

(Xyl)

(
f')

L-

(Fuc;

14.17)

N- ,

. ,

(NeuAc;

u-

N-

14.18),

()

()

). . .

130

, (

N-

0(.

14.18).

) .
N- .
(. .

42).

,
( ) .
f' f'f'

14.17.

-L- (6---L-).

Advances in Carbohydrate Chemistry. Academic Press, 1945current.


Collins . . (ed.) Carbohydrates, m and , 1987.
Gook G. . W., Sloddarr R. W. Surface Carbohydrates of the
Eukaryotic Cell. Academic Press, 1973.
Ferrier R. J .. Collins . . Monosaccharide Chemistry, Penguin
Books, 1972.
Hughes R. . complex carbohydi-ates of mamma1ian
surfaces and their biological roles. Essays Biochem., 1975,
11, 1.
Lindahl u., k . G1ycosaminoglycans and their binding to
biologica1 macromolecu1es. . Rev. Biochem., 1978.
47. 385.
Pigman W. W., Horlon D. (eds) Carbohydrates, Vols. lA
and 1, Academic Press. 1972.
Sharon N. Lectins. Sci. m. (June). 1977. 236. 108.

15

I I

. :

- ,

1)

2)

, . ,
, .

1.

; ,
.

2.

. :
,
.

1.

, ,

.


, -
.
.

, ~

.
.
()
,.,

. ;
lJ

.
,

: ,


.
.

. :
.

. J'l: -

2.

(): ,

3.

: ,

.
.

. .
, , ,

( ).
(. .
,

28),

(),
,
n.

, ;

ol'l ,

( ).

. ( -

152

15

) (

18:1;9

118 18:1

).

,.

10.

CoI9,C18:1 ~9. 18:1

'" 2
J
4

.,

;, 2 2 2 2 2 2 2 = ( 2 .,

- (
).
- (, ),
- (,

2),

J('; ( -

9;

9).

(l

15.1.

). ,

1).

CHJ (CH2 .,=(2 .,

-.

3- -,

. .

15.1.

( )-m-.


. ~ 9

10; 009-

, -.

(.

,IO

15.1.

. (R,'

ro 9, ro 6 ro 3)

-m, 6 9.

15.1.

15.2)

) .

. (.
) .

. 'J. ,
-(20-)- . -

<1

.1

- ()

fl

( 80

).

( ).

()

10

()

, . , ,

12
14
16 }
18

20

22

24

, , ,

1, OI' r. lC .

153

II'lVJluu ll.

15.2.

ll ,

lC

lItl

(..1 )

16: 1; 9

007

..l-

u-~-

II -9-(~

18: 1; 9

009

.
.,

18: 1; 9

009

mpallc-9-0IC-

22: 1; 13
24: 1; 15

009
009

lC

ll-13-

u-15-

r Ol

( )

18:2;9.12

006

-9.

. . .l

12-0

'lwf

( )

18:3; 6. 9.12

18:3:9.12.15

006

ro3

-6.

( ).

9.

12-0-

'lwf

12,

.1I1

15-0lC

-9,

( )

20: 4; 5, 8. 11. 14

006

-5.

B'\fece .l

8. 11.

.l0.

14--

; !\l -

If

( )

20: 5; 5, 8, 11, 14, 17

ro3

-5.

14,

8, 11.

17--

22: 5; 7, 10. 13. 16. 19

003

-7.

13. 16,

10,

19-lC-

( )

22 : 6; 4. 7, 10, 13. 16.


19

ro3

-4.

13. 16,

. .1.

7, 10.

19--

().

. ,

(),

cTaQH.iIH (-l) ().

) (.

15.3).

..

, ~
10

20- ()
(. ) :
(.

15.2).

, ,

in vivo

-" 2 ~. u
. -

15.2.

2 ( 2 ).

154

15

.,~coo-

18

15.3.

,"

, , . .
, - (- 2 )

1200

.....

:u-

9. j

,,

... ...

(R

" ....... ,

.....

" \ )

~(R

F-

(.

..-

_-

...

15.4).

'"
1100,
...

.
I
I

"'"

.
-

15.5.
18: 1) (

(~Q.
).

; .
.

15.4.

r .$.

, ))

120.

,
, , '

. r .

))

-.

, .

) .
u-- "

,
(
).
.
.-

.
r
,
.

, u-,
, , :

.
,

. . .
-
.
-
.

, .

;
. , ))

. '
,

-,
,
.

-,
-

(.

, ,

Q -

, (-

15.5).

llIn

I6 1 ).

155

.I (. 15.27).

11
--- 2

1.

--,

15.6.

.1.

11

2 -

'5 '- -_2

' 2 ---\1 5

15.7.

11

- -

17 5

I.-.

. .
.

12

,
;

18:2,

15.8.

1.2-<t.1.

sn).

3--s- (.

1.2--

15.9). l .
1 3 .

. .

.-

: , u

I (r)

u .-.
-3-.

-l-.
u

-- -

l . ll

15.6,

R- .

2 -

15.7

u u.

. .

11
0- -,

15.8.
.

(lUB).

(lUPAC)

..1.

,
.

15.9.

<l-.- .'1.

156

-,

/5

1)
i. 2)
. 3) . 4)

11
1 2 --- 1

11

2 -- -

8)

+/

11

---- - -N-CH
2
1
22,/

,5) ,6) .7)


21

0-

'~--------~~r--------~/

,....

IS.12.

3-IJ:

.
.'l (.

15.10).

11

11

! (.

-02

15.12).

2 ---

R ---
2
I

J1 ()

11

--

(.

15.13).

0
PIIC.

IS.IO.

lll <i.

0111

2 ---

11

.
(.

R 2 --0--.l

1
11
-- -- 2 - NH
2
1
2
2

15.11).

0- ''---------..v,.------..J/

()
.

. ,
,

IS.I3.

3-)1Jl1;II_

Ji-4,5-

(.

15.14).

- ,

0-

. 11

--0-C-R,
('
2

R2-C-O-~-H
..

2-0-I

I
-9- 0

CH20-~--0--'CH2

11

.
I

--

11

W ----R

----

0-

''------------------~y,.-------------------~/

''-------------------------------~vr------------------------------J/
.

IS.II.

l"Jlu (rJI).

Il{' .111n

u .

'

21

11

, . .

11
2 ---

A2-C-O-~H

(. 15.17).

11

-----

, .

'~------'Y~------~/

A2-C-O~CH

2-0-~-0-2-2-N 3

.'l.'J.

15.14.

--=- 1

I
I

?t

157

0- ''-----.


.
rOK (.

15.17.

.1\.1 (<J.1-..13'Il).

q)

15.15).

,':I. .

88

'

,
, (
15.18). u .

,

11

1CH~O-C-A,

11
+
NH 3
- 0 - - 0- CH2-~H-COO-

(. ).

"
1
A2-c-o_2~H

1,

,~

15.15.

_____________

~,--

___________

~,

'~------------~~----?-------~' ~

"

3-JlIl.

-( 2 , '2-CH==CH-CH-~H-N~

~H2 .. &

... { O=~-O-

. ';Iu,

0- - -N( )

(.

15.16).

15.18.

".1.

()

,
.

15.16.


.
,

10%
.

, .

. -

158

/5

( 2 112 -

= -

1.

- -N -- I- ( 2 1 2 1
,
"w"


'

" &:"cn
_
(. ,88 )

. 15.19. "t (1 , R

= S02-:i)_

= )

,ll (_

I:' .

:!4-
. (. 15.19)
(
),
.
, , J;I
;
.
,
.
r,
.

14).

(NeuAc..

. , -,
.
,

G ; , ,

.-

(ganglioside),
(

3-

NeuAc.

G M ).

15.20. G M

.

: -,
..


.
' ( ).

, ( ,

), ~)

(D).

15.21.

-
,

),

, .

G M1

'""- tO- raJlaKTO~a(I


rQ:)t1)
NeuAc

N- l~"3",-

Cer - GLc - Gal- Gal NAa - Gal

NeuAc
.

15.20.

Gm.-II (.1,1)_

159

lCll Jln

18

12

17

Fl ""

:1
4

15.21.

(, ,

8Fl ""

- ).
.

15.22.

u .

() .

13

19 18),
17 (


).

,
.

( -

).
.

(3), ,
,
(). 5- -

. ,

, 5- -

u-

. .

-
(. 15.22).

C 13 '

-.

et

, .

MOY~

-Of (. 15.23).

, -

-,

15.23.

; -

mu- ; -'-u
.

160

/5

I
-=-=-

15.26.

, n

15.27),

(.

15.24.

(. .

54).

,
.

, d.
3--5,6- (. 15.24).

, , , ~
( ).

()

(),

(.

15.25).

in vivo,

( ).

(ROO", RO',

"),

()

) (.

Cs

15.28).

,
.

, ,

1) : R"

ROOH

2)

+ ()+ -+ ROO" + Jf-I)+ + +

1llle
,

ROO'

R' + 02 -+ ROO'
+ RH -+ ROOH + R"

..

, ,

3)

(. .

ROO' + ROO" -+ ROOR + 02


ROO' + R' -+ ROOR
R' + R' -+ RR
ROOH

27.3),

5-

(.

15.26).

(. . 126)-

( ):


, -

15.25.

r.

15.27.

(9s -).

161

ll 'ln

..

~------

. 15.28. . . ,
,
, ~1r {J) 3 , , l {J) 6- .

..

(<<-)

6-

(),
. ~-

(.

15.29).

1) , , 2) (

. -,

) ,

. .

, -.

, , -

ROOH,

()

, ,

().

in vi-


-,

(. . 126),

(02" ),

ROO'

, ,

-,

, .

' .

in vivo

r, -

(.

15.30).

. , -
;

- ,

(.

.1

15.31).

.
-
.

6 1573

15.29.

-.

$l ", ,

162

/5

' + - - - -....... + '

' + 1'' - - - -....


~ +
~I

. 15.30. l lt'
lt' () It'
lt' (ROO).

-...

ru

~t

)/-=
CHZ
4/ -

CH z

~
---

CHz- z

'"

PIIC. 15.31.

It' lt' -lt'.

Clr(l

lt'
.

15.31.

lt'

It' ( ); lt' .

, ,

,-

, ,
,

(,

).

(. .

. ,

Bnne ;
m

, -Q,

170-225 (.

204) ,

15.32).

. ,

J)ltI nln I---~-__

",


,
.

rnn~1

80~l

~l cno

, ,

.

onn

-i

8 (. 15.32)
.

(2: 1).

1,nnn

1,2lnnn~1

OInnn~1

~
~
-------

fl

:!8.
)(

TO'lKa
l

:-. ..

i!
~L.

~
~

nn~1

1I

15.33.

lt' . It'
lt'-
It' ( 80:20:2 ).

163

'l

"-

--/}

(lt ....)

~----~y~------~

e

()
...

...

111111.=-..
l~~JJ~:

cn

80-", 3loC

8
.."

. 1~.34. , , ,
.

.
II

. ,

, ,

, .

IY


(. 15.32).

,
,

Jl) ,

~ -

Ibl

/5

mm

),
( ),

.
,

(.

15.33).

, ,

. ,

, ,

(, ),

) . ,

(.

15.34).

, , ,
.

, I
( ) - (
).

(. 15.34).
- l' ,
,
- . ,
,

(. .

42).

.
,
,
.

n. -

Christie W. W. Lipid Analysis. Pergamon Press. 1973.


Frankel . N. Chemistry of free radical and singlet oxidation of
lipids, Prog. Lipid. Res., 1985, 23, 197.
Gunstone F. D .. Harwood J. L . Padley F. . The Lipid Handbook, Chapman and , 1986.
Gurr A.I.. James . . Lipid ihistr: Introduction.
3rd ed., Wiley, 1980.
Hawthorne J. N.. Ansell G. . (eds) Phospholipids, Elsevier,
1982.
Johnson . R .. Davenport J. . Biochemistry and Methodology
of Lipids, Wiley, 1971.
Sevanian ., Hochstein . Mechanisms and consequences of lipid peroxidation in biological systems, . Rev. Nutr.,
1985, 5, 365.
Vance D. .. Vance J. . () Biochemistry of Lipids and
Membranes, jmijummigs, 1985.

16

,
:"
;

.
(.

1.

16.1).

, ,

. ,

. -

, ,

2.

J:~
:-

es

-----te
..~

~I,

.,i

l'

~I, ~I,

. .

.:8'

... 2""0-

t~

'\

\ 1,
~-s.

. 16.1. .
,," (2) (- );
,;I . 3BeDM .

166

,
(<< ),
.

/6

yr ~

(.

16.2)

, .


. .


,
,

. ,
,. (
)

~ (, , );

.
(),

- v

'

; -
2 2 ;


8 (.
17.2). ,

, (

) .

~'"Kor...

n38
lO3Q
:8~1

n3008

1 . .t---~L~~~_-..
~

~~/

~.,..".o

L
~

:-8

-.-

~l Cn~1

~-li

)/

.,q~~

01[

"ctJO""

2,

16.2.

1.

( ),

.ll

2.

1.

167

- lItl

+ 2

( )

(2),

~-,

; , ,

2.

3.

3.

().

4.

, .,

(,

).

, -

(.

16.4)

..

(.

( ),
.
()
,
, . .

16.3)

de novo - (
)

.
- (..),

).

-, ~-,

() .

~---._ ,nn
()

IZ

-----------~~~-

~l Cn~1

88
ll88
AMOKCnOT~1

8n-

883

~
KeTooBble

2
.

16.3.

. l.,
. 3- .

rAatlQ

168

16

88I

1 / n

,
n08~.
"--~_--"""~,cn~,,,,,-----....ooj.~830Clt.
~

n.0~.

88

08...

~,

(n)

~e

~~

n-

!f.

~~

~~

....~tf$

2,

. 16.4. )'1.

(, ).

.
,
(]),
().

2.3,


()

( )

, , ,

1)

().

:
-

"

' (,

2)

(, ),

(, )
.0
.

).

(,

;

.

, :!.

~,

, .

.,

~ .

(.

16.5).

50%

; ,

, ,
.

II (.

16.6)

_1 ~--
~---.-:-:\ ~--

. 16.5. " .r If l. J1 ,
. . . .

..

non

.~~~==~---------

W8'l

. 16.6. ; . --
; - ; - ; -- ; --
.

170

16

~~,

'.

"

-1T~~'
..

nr.

~---_/

~.

)"

.....

_---/

. . _ .:

~. ,.. 1U"Z

. . .: . :i~

I~~ \8 :: f"~
:."

!can,_~~)
.... .'

:-....

:~

.... KMCnOT'"

"

.~:.:.:.

...

cn",

Cyin~r\T~

../

.. ~~~~.>
". : '. ~

16.7.

l- .

-+ :; +-+
.

() .

( , )

()

~ '

-.

( ).

, 2 , .


: 1. .

, -

OJm .ll

,
n ii ().

.

2.

171

, ,
,

, -
.

().

),

.
,

, ,

2.4.

;
.

, .

. .

16.7

, ,

, ,

.
,

, . -

(. .

13); -

. .

17

I:

( ,
)
, ,

;

.

( -

- S - , l ),
. ~

, .

u.

.
,
( )
,
; ,

,
, ,

-,
-

-
-

(),


, .
u, n

CO~ u (.

17.1).

u,

u
:!, , , .

po.'IL.

(.)

()


l, u

yr , . u

u-


.

; u
.

17.1.

; r
.

, . .
(. 17.2; . . 13).

llIIU fl: m60!l1l1.' ('mfl_1-

r8'

II

173
_1

~l/
2


r--_ _ _

----,(C)~ 2


I(",

( 4 )

2 "'"

\
8

( )

~- AbIXATEl\bH~5I
- 8

"

-0 - 6\tf(r"i!t
q:>.

17.2.

- - .

-- , 2 2 ,
(2). 2

11

- . Q-- ,
-, --, -- .

174

17

. ,

- (. .

,
-

. ,
. -,

14)
(. 17.3). , u-

.

. ,

NAD+ -,

NADP+ -,

. ,
,

ND+- :

+ NAD+

+--+ (

) +-+ -

+ + 2 -. +
CoA'SH

(),
2

F +-.
:

u- (
), -

~ u---r--+

~
z

. )

.
;
.

,
:

, -

(1975

Mg2+).

.), 11m

(, )

, .
-
, .
(.

18.5):

-
, , NAD+, F ;

- -

+ NAD+ + CoASH -. -

+ 2 + NADH + +.

-,

(. . 186),
,
(-).

- ,

( )

(--):

' .

Mn2+ (

, 14 ,

-.

-,

\.
HzO

-;
,

+ NADH + +.

,
,

17.3)'


(.

- 2
- - .

+ P + GDP +- -. + GTP +
j

CoA'SH

175

.wU : ma60.w -
~

.. ..
'L'

'

--S-
-

8 l
.\

. ...... .

Ho-

"

88

11

--

CoASH

6-

-- -

88

2~OO-

---

~~~~COO\- """

62-oo~
L-

_~
!'- 2

Fe 2+

--

000-

- \

--

11

:- --

....

--

- ...

u-

FAD
)-8II1II\,....---.....

" , , 4t

H~-

---

~K~P
M 2+

NAD

CoA.SH

~-

NADH +

GDP+P.
I

.i.

~COQ

2
O=C-S-
-
-

+ ~~ ~ 3OQ>lPl-

_por...

.]
000-

,--

I
=--

~Koo- \ ~ can

~1 2
Mn 2+
=--

. 17.3. ( ). NADH F ADH 2


. - .
, -.
, 2 . .-,
. , lC.
- . ,

2 , lC

, ( ) .

.
(. . 20.1).
(Greville, 1968). ()
,, ".

176

J7

GDP (
IDP),
GTP (ITP). -

1<,

-- ,

YPOBHe~

NADH

(,
)
.

NADH

. .

GTP,

ITP.

GTP

+ +--+ GDP + .

COA---

;,

(),

(. .

290).

F ADHz.

(.

17.2).

.
,
,
:

NADH

NADH


D
(. . 13). FADH 2

+ F +- -+ + F 2

F D 2

';

(. . 13.6).

. . ,

NAD+.

(Fe:S)

F D

. '

- . ,
J

12

(.

17.1).

,
-

d ,

()

17.1.

-0

+ 2 +- -+ L-.

L- ,

L-

NAD+:

+-NAD+

+ NADH

+ +

NADH

NADH

+--+

FADH2

NADH

C;l
, -

12

177

,1I : u' (mu-

, "
I

11

'

11

,-

(FAD),

1)

- 2 ----"N- 2 - z --

17.5.

2)
(NAD),
:
, -

( ,)

3)

,
-.

4)

, , -

-. (
. .

121-123)

(.

( )
,

(
),

4' -.

17.4.

l'

17.5).
4' -
(.
17.6).

Il

, (
).

,
;

4' -

3'-
. ....

1)

(-,

, )

2)

(,

).

(. .

53).

18.5;

- .

(. .
)

CoA'SH,
S-

0-

C-s

N ~C..... - 2- N+q
1

C~

11

./" "":N'"

0-

11

11


, ;

2.-

------

=- 2 - 1 -

, , .

2---

6-

, .

17.4.

. --rp
.

,
.

178

/7

, ,

88

V:-

ATP

f"-ADP

=-1

'

-C-CH-C-N-CH-CH -12
2 2
<;>

(. .

0-

196).

4-8

GTP

(.

+,+
~H ?
~ ~O(

y---N-:r-N--2 -SH


o-f.-o-

17.7):

+ GTP -. +
CO~ + GDP.

0-

4-8

1'-ro,

I I
I

2----N----N- - -SH

~ 9 ~

. ,

-. +

=--

-.

0-

- ,

4-8

, .

ATP

~PP.


'

-~~-~-N-tir-~-N-
- -SH
121
22
22
()

NH

o=~-o- N~)

V-

ATP

f'-...ADP
~-8

388

tJO---<~J
~- } -3-
22

17.6.

+ ..- -. + ,
+ ..- -+ - + .

CH----N--::-_N- - -s

~~~
~ <j>H ~
~

22

N~}
~

-o-J--o
-

. :

poocl -~-oI

NH

121

, ,

-- .

8"

, .

O=~-O-C~
1
20

, , ,.

, , .

, ,

(1-

.; , .

-;
(. 17.7). ,
,
CO~ ,

-,

.

IIIIIU : u_w u-

179

----------------------~

~~==:::::!.~ -

--------~

}
10

----------~.

-------------

-----------------------...

17.7.

.
.

- - - -.....~

~ -

~~~~~' ~--------+tI-l~

17.8.

2
-

----~

. . .

23.9.

/:'(/

180

) - ,

- (. .

(.
-,

20.2).

- ).

,
,

+ -+ - +
+ + ,.

22.

,
.

17.8)

/7

: -
, ;

- -

. D. (ed.) Enzymes. 3rd ed., Academic Press, 1971.


Good'in . W. (ed.) Metabolic Roles of Citrate, Academic
Press, 1968.
Greville . D. Yol. ], . 297. ': Carbohydrate Metabolism and
Its Disorders, Dickens F., Randle . J . Whelan W. J. (eds),
Academic Press. 1968.
LO'enstein J. . 1. 1, . 146. In: Metabo1ic Pathways, 3rd
ed., Grenberg . .. (ed.). Academic Press, 1967.
Lo'enstein J. . (ed.) Citric Acid 1: Contro1 and Compartmentation, Dekker. 1969.
Lowenstein J. . (ed.) Citric Acid 1, Yol. 13. In: Mcthods
in Enzymology. Academic Press. 1969.
Srere . . enzymo1ogy ofthe formation and breakdown of
citratc, Adv. Enzymol .. 1975. 43, 57.

18

. (,
, )
. .'1--

; .
.

( ),

11 (

).

.
.

~I

- TO.11.KO

, .

lll ,

"J

~, ~ .

8 II

8 :

: ,

ri.11. lf

. 2 2 .

r l1

fl> .

D .

"J .

,-

(.).

(,

); "

(.

18.1).

'

NADH,

NAD

. ,

HCKoropbJx . -

, .

182

18
:

M g1+

-D-

+ -+ -D--- + .

, ,

( ) " ;

, ~

~ .
,

0-, -

; ,

-----NAD+

).

NADH

++
v

~
. _,;::"",....:;;..._

18.1.

22.7).

100 Mf/lOO

(.

-6-
(

, , ,
) (.

18.2).

-6-
,

--.

- -6-:

,
, :

+ 2 ; -+ 2L- +

+ 2 2.

(.

18.2)

().
,

- - -6- ~ - D--6-.

Mg-ATP.

: ,

. ,

,
:

D--6-

-+ D-

-1.6-

6-.
;

(-I)

-I,6-.

-I,6-

(-I,6--)

: --
:

D--l ,6- ~

0- .

-6- -

TaK~e .

uu

183

ullu nm

0-1-

H~H2 ~:i:::~:::~:;:::~:::~~.~~ ;;r~:~====~=~=~=;=:=~===~-~~:20

----

-- 38

~-1,- 8

~~n

~-3~

1,3-

11
-

- t-o~
I

2~

:
I

i!

--1

--

11

r-:::
:

Mg2+

. I
-
11

nl>

1
I

,1

,
1

T~NAoJH+
-

", R

<}+ ~:':--I
Gr1""
-

%02

I
1

I
I

JAO+

')

I
\..
= ~..t;::::::==-===~-=:;::::==:::-.
I

()

(,

--
1

l(+)-

. 18.2. _ : --(- PO~-); Pi-HOPO~--; -r_

1-3

.
4--6- -3-_

184

/8

; ,

NAOH
, NAO.
NAD.

3-

D--3- ~
.

-3-

1,3-


l,3-,

-3-:

+ NAD+ + Pj~ 1,
+ NAOH + +.

ND-


Pj ;

1,3-
18.3).

-S- (.

,
-

1,3-,

1.

D--3-

NAD.

, 3-:
1.3- + ~
+ .

r -3-r-:

3-

.
, .

SH -

,
, ~)

-3-.

-=

--

2"-

_---

-3-

..---_.........

s-

-v

?-
=

--

---Pi

CHi""O-
1,3- .__----IL---_.
s-

~=O
I

- -

NAD+

2"-

s-

NADH
I~
+

==
I

--

.+

2 --

PIIC. 18.3. --. -r--. rJl _


- S-rp lIO_, , , Jl ..

-3-,

(P j ).

185

1-

. ll

3-

. .

,,:

3-

900/0

2-

(, - ,

2.3-

, )

(DPG):

3- +-+ 2- .
, ,

,

;


..

.
; ,
,

.
Mg2+ n 2 +:
2- +-+

+ 2 .

6

;
.

. ,
. .

. ,
( ),

. ,

(),

. ,
.

, . . . -

-+

+ .

NADH

NADH

2,3-

,

, ;


1,3-,
(.

18.4).

1,3-

2,3-,

3-

2,3- (

).

+ NADH + + +-+ L-l' + NAD+.

NADH

NAD+,

-3-

. , ,
,
(.

18.2).

.
,

, .

2,3- u

,
, . .

. .

2,3-

ror:

(. .

6).

186

18
.

-- ~--- 8

--

2--@

NAD,

..-3-

Pi~NAD.

I er-3- 8 I

+ NAD+ + --+ - +
NADH

..

+ + + 02

NADH +

--

--

29

-@

1 3 8

, .
,

---

- ,

2--@

2.3-

(NADH)

-.

~OO
--

2.3'F

: -@
3-
L __ .....


,
-SH -
;

18.4.

2.- .

r .


, ,

(. .

13.15).

()

j:

-.

,
u

u-

(. .


1
0 2 2
2780 .

174).


; Ji,

i\

, 02 2.
38
. ,
30.5 .
, , 1159
1 ( 41,7%
).

1e


(. 18.5).
,
- .

NH 2
1

~C'C-CH
11

18.5. -

_
2

~N/

+~
,

=- 2 - 2--
I

- r'~--GOO7"
~

JI

C-S

-9-0

- -

- s

I t;::=~===~ -+r( I

+~

t-s

=-

=-

s]

(1

['s

- CoA-S

-s--

IJ
~

r{SH

[.

11

........... S--
L
""-SH

L'SHJ
.

--:=:========::::;----:
FADH

F'~
~

NAD+

NADH + +

18.1.

8:

'" 0,

- 3-

2 NADH

10
,

-2

2 NADH
2 NADH
2 NADH

6
6
6

4
6

2 FADH~
2 NADH

1)

NADH,

30

38

<t , ~<t

\.V

(. . 13.15)_ , 2 1 NADH,
38, 36.
+
. 1 .

..

188

.
,

(. .

13).

18.1

,

.

lass

J. . Disorders ofpyruvate metabolism, Neuro1ogy, 1978,


29, 280.

18
.

D. (ed.) Enzymes, 3rd ed., Vols. 5-9, Academic


Press, 1972.
Dickens F.. Randle . J .. Whelan W. J. (eds) Carbohydrate
tabolism and lts Disorders, 2 vols., Academic Press, 1968.
Greenherg D.M. (ed.) Metabo1ic Pathways. 3rd ed . 1. 1.
Academic Press, 1967.
Randle . J .. Steiner D. F.. Jf,'helan W. J. (eds) Carbohydrate
Metabolism and Its Disorders. Vol. 3, Academic Press.
1981.
Veneziale . . (ed.) The Regulation ofCarbohydrate Formation and Uti1ization in Mammals. University Park Press.
1981.

19

; .

6%

) ,

1%

(. .

19.1).

(.

14.15).

(.

-6-

+ -6-+-+ + 0+ -l-.

1,6- +-+ -

12-18

"I

.

,

.

,

19.1.

70

-l-

(UTP);

(UDPGlc) 1

(.

19.2).
-l-

UDPGlc-

UTP

+ .-l-+-+ UDPG1c + PP

,
, .

, ,

0-

-l,6-:

, -

, ()

-6-

. ()

, ,

l-: ,

19.1)

) -

UDPGlc,

4,0% = 72 r
0,7% = 245 2)
0,1% = 10 ))

(UDP).

ll

327 r

1800 r.
35 Kr.

1.

71

))

UDPGal.

. .
(

10

), , ,
I .

190

L
/

19

(1-+4 1-+6 )

I~

(1 -+4 ) v
I

:8

UDP

+
f:t\ -------,\
8____ cAMP---~1

"8

"

: ()

,
.

( (UDPGlc)

m. etlUU
0IIUR

UDPGIC -

/ P Pj
2P j

-1-

(t)

g'+

tI

--

~-6-

~eKC030MOHO-

~OP

g6'

Mg2+

I I
.

Pi

19.1. . .

. - ;

. lt , . . .

(. .

,CH,~~ .

.o

--

11

11
I

0-

0-

HN:)}

O~N

~'

'''----y----J

19.2. (UDPGlc).

,
;

(1

-+ 4)- (

.. 19.3).

II

mo, lllll (

) (-(I

-+ 4)-+ (1 -+ 6)-
(1 -+ 4)- (

+ () -+ UDP + (6 )+ ,.

-- ]

0-

54):
UDPGIc

191

lrnULW

, ...

0--0 1 -+4-
t>I

,,

0+0 1 ..... 6-

"

14 :
I

1\

14

8.

(1

19.3.

. [l] .

-+ )-;

(1

-+ 4)-I

. ,

II .

14

(.

19.3).

l-

-. ( )

l'-6-,

--.


(. . 19.1)

, , :

( 6 )

; -+ ( 6 )-I
-I-

()

(1

-+ 4)-

, -l-.

, ,

([1

r (.

-+ }-),

19.4).

(<<-11 -+ 41-+ -11 -+ 4)-)


, (l -+ )- .
(1 -+ )-

()

(-(I -+

6)-

); -,

1.
1 (1 _ 6)-
J:~ , 1 ,
1 QJI -l-. ll

-6-~ lClC , ,
J::, 1 ( , . )
f!.

.... }

0-0

,
1 ..... 4-

1 .....6-

;.

19.4.

192

, 19
.-
-

(. . 104).

(. .

108).

/9

. :
-
, ,

( ),

~8 .
.

. ,

(.

19.5)

(.

.
(~ )

19.5).

(3',5'-

; ) (.
19.6 . 44).

(~-l)
~8

()

~-

, ;

I' -

JUOKarOHOM,

8II

I3-
...

10
+

..
-

8-

[~

:s

(11

+
(+ 1)

-1-

A t P - - - - - cAMP~==========~=_ 5'

..

JG

..
cAMP-t----.;..-- cAMP-

n ADP

-1

()
n.
:s

ADP
(.)

~'-1-

(It.).
.

19.5.

------------"""'--.;-----------------------

( - ).
; .

NH

~ )4. ~ .

N~ '/"
1

11

HC~ /, /
-"':N

-,

-.

cAMP-

. ~- 2 + ,
2 + -
. 2 +

yr :!. +.

2 + -

().

,
. ,

19.6. 3'.

5'-I1.10 KI1C,lOTi1 (l1J1I1 .


).

2 + - .
-

(. .

44).

".
,
;

,
-

cAMP- .

ll

, ,
, (.

19.5).

"I-.
cAl\IP-8 2 +
,

2+ /ll ll
.

r
. .
~.


-I .

cAMP-
;

(R),

(), .
R 2C 2
.

-l.

- (. .

-l;

R2C2 + 4 +-~2C

44):

+ 2(R-2).

2 +

-I .

cAMP- . ,
,
(.

19.5).


(. 19.7)

.
2 + -

,
.

. .

(8 ),

i
: . ~, . - (

ll

7 1573

lItI

194

/9

' 1

....

.-

ATP----~l_+__~.

..I

5'-

I--0-:::+=--- 2 +
I
...

(" + 1)

() I

-6-

(" I

+ UDPG/c

-1

----------=--.1

()

19.7.

(11

). Il

1 . ;1;

ll _I.

HaHoMoJlbIlbIC

-- JfJt-3, -4 -5. -

i:ll{.

cAMP-

(.

2 + j.

19.7).

219.

cAMP- .; l10

,
()

OIl ~, II
ll ll llO.

-.

-4

-5.

--

1 l:l ( )

,
UD-

: .

.
.

-I.

.-

. , -

llO -6- ll

II

i )

- -+

4)-

(1

(llO

, .

f'

195

-+ 6)- (

VI ),

(ll
VII ; ll
), .

C10II ).

.[:11'1111 .

cAMP- ll.

:' ~\ \~OMax.
1".lIIO

111

(IIO: lll

ll )

;

( ).

JII"IIO IV (IIO; AIIll)


.

-

.

(
)

llO V
(Jlll Il; I

).
.

(2.5--4.1 %).

.

., Brlm B.I. Some il" errors of carbohydrate


metabolism. Page 391. In: International Review of
Si. Vol. 5. Whl W. J. (ed.). Butterworth. 1975.
Cohen . Control of Enzyme Activi\y. 2nd ed .. Chapman and
. 1983.
/1n . The role protcin phosphorylation in the hl
ntrol of 7 activity. Eur. J. Biochem .. 1985. 151.439.
Exton J. . Molecular mechanism involved in a-adrenergic rcsponses. l. . Endocrinol .. 1981.23.233.
Hers . G. The cOl1trol of glycogen me\abolism in the livcr.
nu. Rev. Biochem .. 1976. 45. 167.
Rand/e P.J., Steiner D.F., Whelan U'.J. ('{i,'i). Carbohydra\e
Metabolism and Its Disorders. Vol. 3. Academic Prcss.
1981.
Sper/ing ., de Vries . (ed<;). Inborn Errors of Me\abolism in
. Karger. 1978.
Stanbury J. . et /. (ed,'i). The ctabolic Basis Ihitd Disease. 5th ed . Gw-ilI. 1983.

Brm.." D.

20

, ,
, .
- .

,
.

(.

. , ( ) .

20.1)


. ,

2)

1)

l,- --.

-- .

3)
4)

-l- .

1)

. , (

) 0 2

2 ( ) . 2

(. ).

, ; ,

, -

. ,

02 .

.
. ,

ITP;

.
()

GTP

, ,

: ,

. ,

, -

'llll nlllnmll "

197

'I

- - - - -...... r

Io8r--I .....
1 - - - - 1... rI8-

NAD+:r

NADH + +

- ~

NAD+

NADH ++

,- ~~~~";

~~~'"'\J-r.:::":-~:-:-=8==-=~:-:-:-:~:-1

~TP

'.~
2ln_~'

8I8

TP+C02
11 V888:n8 11

W
.'" ~ Aetn-<:
Mg2+

+ -

NADH +

NAD+7

4--ff-----n

'"

\
-r

.. .._

........

O-~,,<~
~
. 20.1. lC . ", _1 l1 '(
(. . 17.7).
rII. . Jl ;I . J' ,1-

. I- .1 J _l (1 . .). ._l- .-. .

198

20

Oll,

-.

2)

--,

Q--ll.

, -. 2 .

-I,-

1,6-. -

D-- (.

--60

20.2).

:!

, ,

CoA- (.

. ,

--

--

3)

23).

II.

L--.

-6-.

--, J10

-.

BI2

4)

l-

812.

OllO ).

ll (. .

19.1).

20.1.

C'!I-

. 7-

;
,

. (-)

11 . , ,

, .

-3-.

. ~ -

~,

--

+ ,

-- I

- S -

-
D+

co-s-

[).-

, -
""'M-----------'-1
CoA-

II-------....

....

~H2

CoA-

1"

~H2

20.2.

]
I

812 (-cr-

s-

..

CO-S-

L --

,llu nllll

3-

NAD+

-3-
.

20.1.

199

8:


- -l.- --

(
)

(.

20.1).

--

(.

-,

20.3).

HaT~

ll.

(.
20.1), .
,
- .
,

,
N' .
~ -

20.4).

. ,

(.

, . Mg + - (
).

~-
CoA-

J3-.

, , ,
,

, ,

(-) ,


. (

) ,

, (

: .
,

(,
-
).

--

2 .

--

. ,

-3-.


.
.
ICa
.

J3-,

13-

-3-

-~,

:

H-N

11
..... ....

NADPH

---

2 ........ - ( 2 ).

+.


, : 1)

NADPH

, ;

20.3.

N-H

+2

-- + NADP+ -+ 3 2
-- + --

2)

200

20
0-

;,

--

11
- 1,. ....

11

,.,

HN

M g 2+
i~-,

NH

I
I

,,.

1
.

NH

".

- ( 2 1.

I
I
-- ~ -

--

=0
I

,~

HC-ICH")4
,.,.
I

0_.
I
S
-
CO 2 --1
=

~ = -

-N

-N

I
..
:~,

20.4. J1 2 --

0-

, 8r8-

, 6-.

. ,
-6-.

688,
ND+- .

100

-6-

~;

--5-. ,
,

3--6-.

Jl

()

-5-

. -

5-8-3-n88

-5-, . . .

-5-8"88

NAD,

3:

. ,

5- -

NADP.

-5-.

888 ,

. -6-

. l- 2- ,

-5~.

, ,

-5-

. .

6- ,

: 88

.
(

8888 (.

20.5).

8 - ),

-6-

ND-

6-88,

mo-

Mg2+.

. ', . .

6-

6--

-5- -5- "

ll nmu

201

-7-
-3-.

. -S-

>.


-4-.

-6- -3-.

1-3),

-7-

-3-;
-6-

, ,

-4-.

-3-

11

-<;-

~
I

NADP+

NADPH+H"

Mg2~'

,
--
~"MA:;.. Ca2'
--
,
-"

HO-~-H.

-- ('
-- -6- - -- 11,

-
I

2 - 0-

'
1

--
\
'I\IA ,.
I
:--..
.. --

2 Mg2:

2 :

- - -

--

1 --

t3-~r0-6-

2 --

6-

6-

6-- ~91;r:~~+
..

NADPH
+'

-6-- ,
I

11

--
I

..

- -

-I -

--

--
I

2 - -

,--

EHlbH8R

- -
,

--

2 - _

30 -5-

3--6-

-5-3-
2
I

--

HO~C-H

H-~~
--

--

2 - -

- -----.J

H~C-OH

c~,-o-
-5-

HO-C~H
I

--

-5-

--

,--

~ATP

-7 -

M g 2'

-_

,.

----

-~
--

--

" 2 --
-3-

-I ----.J

2 --

20.5.

-(-

PO~--); R--5--l-_

202

20

H~C=O
I

H~C -
I

' --

-3-

OCHzOH
I

HO~C-H
I

--

=
I

~_ _ _~===~=======I~_ -
I

H~C-OH

- -

I
H~C-OH

- -

-=

",-0-3

CHz--

-7 -

-6-

--
I

CHz --

CHzOH

4-

CHz.OH

=
I
--

- -
I
- -

II

--

--
I

-z

CHz. --

Mg2."

-5-

CH z -o-~

-=

--

- -
I

CHz.--

-3-
.

20.5.

(). .

6-.

. ,

D ,
-l,6-.
. 20.6.

NADPH,

, ,

)
. .

NADPH,


NADP: NADPH.

, .


.
. N , .
NADP; ,
2 ,

. ,

-6-

6-

(.

20.5).

-5-.

PRPP. (.
. 35).
-6- 6-

, , .

, -

nmm
-6-

C6~

C6~

-6-

-6-

NADP+

r-

-6-

<;;

NADP+

6--.:

NADP+

~NADPH+ +

NADPH + +

NADPH + +
6-

203

6-

6-

NADPH + +

NADP+fi+

-5-

NADPH + +

-5-

-5-

-:ij j:!51 1 -(51


-5-

-5-

-5-

C5~

Cs

-- - 7 -

jI
-6-

-4-

-6-

--

! -

r----------,

1/2

~:l~

1.::::=:.&

1/2 -6-

L -_
_ _ _ _ _.....J

-6-

-6-

1/2 -6-

. 20.6. .

204

20

IO-6-

-6-. ,

;
NADPH,
,

,-

, , ;

NADPH
(G-S-S-G)
(2G-SH),

(Vicia faba).
~

2 2 ,

. ,

n:

G-S-S-G

+ NADPH + + -+ 2G-SH + NADP+

,-

;I8
JC

2G-SH

+ 2 2 -+ G-S-S-G + 2 2
Jl8
JC88

ll

2 2

). ,

Krebs . . Gluconeogenesis, Proc. R. Soc. Lond. [Biol.], 1964,


159, 545.
Landau . R., Wood . G. The pentose l in animal tissues:
Evidence for the classical and gist the .. L-type pathway. Trends Biochem. Sci., 1983. 8. 292.
Wims J. F. critical examination the evidence of the reactions of the pentose pathway in animal tissues, Trends
Biochem. Sci., 1980. 5. 315.
Wood . The Pentose Pathway. Academic'Press, 1985.

21

Ix

, ,
.
, .

, ,

.

(. .

,
,

.
. 21.1. --
-I-,
(UTP) "

(UD-).
UD-.
, ,

, ,
, .

19.1). UDP-

, .
, NAD UD-,
UD-.

UD-

( <? ).

Ji .

, (
)

(. 33.1 ).
ND-

( )

L- (.

()

21.1),

Q ,

3--L-,

J:l,

L-.
D-
, ,

21.1,

L-.

L- D-;


-- ,

D-

-5-,

ND-

L-

NAD.

206

21
H':C-O-UDP

..!-
'~

H-~-?H

--

-9- 0
-

H~C-O-,--~~-----, H':C;:-O-UDP
'~ UDPGlc-

H-~-OH

--

H-~';OH

2 --

-D--

+2'

11

(UDPGlc)

--

8'

-1-

HO-~-H

~AD'ANADH -

UDPGlc-- H-~""OH

I (:( ,

--

P~

UTP

--

( ,

-9-0

H:O~

nlO.

UDP~

=
1

--

11
1

-- -

..

--
1

=
1

11

NADH +

"
-

)
-

--
1

--

--
1

--L-

L -

NADPH+H+

D- ;-1-

NAD+ NADPH+H+

- -
1

'-

>4

~.~-~-~--.8'

--
1

--

./

- - D-
I

..

D-

- -
1

2
2
'-.......;;.-'----.. . . M9~:'i
AOP~
D--5-

' -? -
HO-~ -

-9- 0
-

--

--
I

11

L-

D-

,-

02

- -

8'

NADP' NADPH +
'-.. )

-;:-

-'

--

NAD"


t
2--L-

NADP+

.!-

11

11

--,

11

-.J

-;:
HO-~-H
I

[2!

..

o=tl
I

=;:

11

- ---.J

- -
I

L -

L-

11.1. . . C-l ;

-- 2-.

207

mJlU' ll

-l.6-.




L-.

,
.
L- .

D-.
-l-.
(. '.

21.1).

. ,

, L- .

L-


. -

,
L-
.

--

.
.


-l,6-

. .
-I- -I.6-

.

, .

--.

Ji,

<;

. ,

.

-6- ,

(.

, .

. . .

, ,

21.2).

, ~

l-.

. (

. ; ,

.;

. ...

.
l- , -.
.

-I-

~

. ,

. , ,

(.

21.2)

208

tt

)1

21

II

-6-

~ :
~

""'--

~-

Dco

NADPH

-6-

7 "\
::".
...

+ +

II

-6-~-------

__________ ~~

-1.6

II

-1-

-1 ,6-

I I

I I

-- ....f----;;:::;;;;;;;::;::;=.;:;:;:::;;:;::;------I~r

I I

2-

11.1.

. . ,

. .

(. ),

, l\10 ,

NADPH

NAD (

9).
.

. .

,
. -

u' u

209

3)

( ).

. ,

UD-, .

-
.

-4 NAD
. ,

UD- -I-

21.3.

1,

).

--I-

, ,

( 4), ,
.

, ,

(UD-)

r,

I-. (

2),

, (),

(UD-)

-I--

UD- UD--

Il Q)

1- ) 1 2 UDPGlc
-1-

~
UDP

-1-

UDPGal

QD
---------....
UDP-r-

NAD+

4-

UDPGLc

~pp~

UDPGlc

-1-

UTP

A'jC~~~Hi::P
:

-6-

. ~-------------------.
I

21.3.

I-6- I

210

21

~ ~

-1-

1. --

\..

--

~
-

'.

I!

\.....J..

tr

tr:.1

N-- ~

.L

UOP- ~
~

'

1-

'8

UTP

N--

'8

..

N--

1-

(, )

lll

N--

~UTP

P2~

UOP-

. ~
NAD+

11

UOP-

9- N-

----I.~
(s )

N-

N-

---

() ,

. 21.4. JJ . V--

VOP-GIc.

- D-N-~_

UD-_


().

IQ,

1, 2. 3

2-1.3.

(2).

l-,

,
.

I-- (

2).

, (

3)

. ,

mu Ilu ll

. ,

211

-;

).

--;

(3)

N-

N--

N(4) NeuAc

,
.

(5)

UD-N-

--

( )

(UDPGlcNAc) UD-N- (
GaINAc); ()

UDPGIcNAc, UDPGalNAc

500/0

CMPNeuAc.

'n

~
() (. 21.4)

(. .

(,

54),

(. .

54).

15)

-)

9-- .
, ,

N-

(NeuAc).

21.4;

(1)

0-

- --.

(2)

D. ., 'n . J. Some inborn errors of carbohydrate


metabolism. Page 391. ': International Review of
Science, Vol 5, Whelan W. J. (ed.), Butterworth, 1975.
Dickens F., Randel . J., Whelan W. J. (ed.). Carbohydrate
tabolism and Its Disorders, 2 vols, Academic Press, 1968.
Huijing F. Textbook errors, Galactose metabolism and galactosemia, Trends Biochem. Sci., 1978, 3. N 129.
James . . et /. Models for the metabolic production of
late from xylitol in humans. Aust. J. . Biol. Med. Sci.,
1982, 60. 117.
Kador . F., Akagi ., Kinoshita J. . The effects of aldose reductase and its inhibition sugar cataract formation,
tabolism, 1986. 35, 15.
Macdonald J., Vrana . (). Metabolic Effects of Dietary
Carbohydrates. Karger, 1986.
Randle . J., Steiner D. F., Whelan W. J. (). Carbohydrate
Metabolism and Its Disorders, Vol 3. Academic Press.
1981.
Sperling ., de Vries . (eds). Inborn Errors of Metabolism in
. Karger, 1978.
Stanbury J. . et /. (eds). The Metabolic Basis of Inherited Diseasc, 5th ed. McGraw-ill, 1983.

22

(. .

9),

, ,

. .

(, ,

, ,

; . .

53.)

.
,
, .



, ,

, ,

. ,


. (

).

. ,
.

--8

. .

-D

in vivo


. ,

.

.

.
,



. ,
, (.... Jl

.
,

--..1.. -

21

ero .

(.

22.2),

,
;
(

,
, . , , . 22.1.
+-+ +-+ D
, -+ C-.

.

, ,

, , -

2+/--.....:G
=+_ _...~..

0""----

.....
__-

.....

-------!~ ~

J.

I]

.. ..

..

~[2]~

__-->G""-::::,_-II.~
@ G

22.1. . . .
I - ; 2 - ;
- ; 4-J1 ; 5-

~14

l:lt{/

J::

, ,

Jl ,

.

,

22.1.

.
, ,



(. .

9).

(. .

43).

.
~
,
(

. ) .
cAMP-l ,

,
,
.



,
.
,
,

.

,

. .

,
,

). ,
,

22.1): (1)
(2)

(.

(3)

(, u ),
. ,
u lI (. .-
).

tl

cAMP- .

, , ,

[.

[ATP]j[ADP] ( - ~ .
22.3) . + I
( - ; .
2

19.5).

22.1.

,
.

,
.

. '

(.

22.2).

, -

, ,

(.

, . . -

41

43).

22.1.

22.1.

215

( )


-6-

f
f

!
!

(),

-l

0-

",

6-

'),

",

)I), Tp l),

11

()

'
,

-2,6-

-I,6-

NAO,

, ,
(),

-,

NAOH,

11, ,

-"

()

"

-l,6-

-l,6-
1),l),30- 2,6-

-6-

-6--

f
f
f

!
!
!

6-

))

--
---

'1 .
11

, .

'1,

-,

216

22

I\

/,."",L ..

'\

1"

-6-..........-1-.

1.

.....- - - - - - . . .

t t. _____

-~.I

....
.
, -

te
I
I

I
L- _ _ _ _ _ _ - ,

I
I

.....!... I

I---...J
-,

I
I
I

' - - - - - - - - - __ ...J

L-~~~~~~~~~~_e____-,

i'-------

fi8838

-_1,_6-...J--==~-...,.

- ",--(.
Js
_
2,6-.

2,6-

.,

.,--.

-6-

~-~--~-~-03--~1.~6--~

(.)

\.

,...-_;:::,_ _::;;...._...

UDP-

..... _

8-

.8

NAD+

tNADH +

NADH +

-:~:

1,-

Q- GP ~

...

NALn
+ +

1------ .

....

.,

.,8

88

...1I(
.....------"'.I.~

.,.

~K"H"-CoA7

,,- ""';;&&

- -_ _ _ _WOI

___""""'!." } R@.. . ..
!' (!) .. . .

, . .
R .R

. 11.1. . . _
.
-I -l.6-
(. . 22.4)_ , . . . )) ..

.'1 <'

[NADH)

217
()
()

. _ - - -

g2+ ;;.+
. (

. 22.3. rJJl (). d


. -ry r ; -r
.

,
; ,

, . ,

,
-

, r ,

, .

II

, .

,
, Q

[-]/[],
[NADHJ/[NAD+] [ATP]/[ADP]
. , rr

, lI , ,

Sl CJl

(.

22.3).

~ _"

r ,

( ).
mo

II , .

mo8

d> -

; ,

cAMP- ll;
n

, r.

8.

"

218
-2,6-, ,

.
-l

v.

,
,

.
,
,

-.
,

~ r.

, -,

.
,

-,

. ,

.
.
-l
,

, ,

.
-I

6-.

.- ,

,

,

, (.

22.2).


, ,

,
.

(-I)

,
.

. -l
.

-2,6-

-I
-I ,6-

2,6-.
-I
-6-.

-I,6- 2,6-

-I,6-. -

2,6-
-6- (.
2,6-
-6-,

22.4).

-2.

0-

2,6- )

-6-

( -6-,
i,

). ,

+ AMP ..... 2ADP

- ,

-2
-2,6-

50
,

. ,

-l I

-2,6-,

-l,6-;

n-

u m.lU1 ,l

219

ADP

2.6-

-, I . ~
.

22.4.

-I.6-: -2.6-

:II

i.l: -2

.III: Ja

1 jlr1

rlC'll:ll1I

} lu-2.6-fi:: 1\)'\1.

r~ /U-2.6-ll~ I.HLl: -I

-I.l)-!l

6-) 1()-I--

6--2-l<. .l.lI: . tll1 .l I.lt 1LI \111 r pC.1KLI\III.

-2.6-; (. 22.4)
-l
-l ,6-,
.

-I.

-l

I (. 22.5).

. .

cAMP- .


(. 22.5)

OJI

. uu

).

'l.

(.

19);

cAMP-

-I . .
22.5).

. ,

(.

5-,

(. ).

, -

. , -

220
8

(~, ~'~') - -....

- - - " " '...

22

5' -11-___~ 1

----_

(~)

38

,
,

\
-

-1

38-1

I
838

38

.... _--- - ' "

38-1

cAMP- .

22.S.

, ( ). .

( ). u -

{,

ul-

2+
. ,

11

cAMP-

'

,
:

j-6-;

l/-l,6-; /
j
; /.
,
,
. .
,

. , ,

-6-.
,

.

221

(. .

18.3)

,
,

. , .

(,

in vivo,

,

,
.

,
-

NAD),
ADP , ,

. ,

.
,

.
(. . 21.2

. ,

21.3).
.

- ,

(.

22.2).
(1)

, .

--

; ,

(2) .

ND-

. , ,

ADP

, ,

, -,

HeI"O .
.

NADH.

22.6).

Te'Ja

[NADH]{[NAD+].

..

(.
.

. -

.,

IO3-8-

7"i

~
An

1,

..--....r
~

8ior,

.. .8
...

:r

\ --------84

..,--...

r8

lOk30-&

~ ~'

8., 4

., V8l

I~~

-_.-.--.-----_... _....

",,;

AnI....

' - _ - - - - - - - - -

22.6.

( ) -

222

,
, _

22

. -'

, .

, .

. ,
-6- ,

, '

, .

, ,

.
, -6-

(.

22.6),

- ,

.
(

) , ;

. ,

(.

. .

. ,

80

100

/l00 . , <? ,

60--70 Mf/lOO .

(80--100

/l00 ) , -,

120--130 .

/lO .

22.7);

.
,

150

/100

40 Mf/lOO

60 Mf/lOO

. , -,
,

, ,

() ,

.
,

100

---------------------------

II

II

100

,
lQ -

300

400

3 ..1 , /100


. ,

200

22.7.

. - J (0,9 Mr/lOO ), -l

0,05

'-

(180 Mr/lOO

).

223

6-

- ,

, .

. ,

, ,

( ).

, ,

, , ,

; , ,

; ,

. , ,

, ,

. ,

(. ).
lUIJI ,

, . . .

, (

,
. ,

) , , .

. ,

, .

, ..

lC ,

.
. ,
,

-.

(II-)

. ,


(
) .

.
350
- 1

,
,

. , . .

(,

, ,

, . .).

170-180 Mf/lOO


, -

224

22
.

~
~

, ,

, p~e

L.

.!

, , p~a

s
&
:i

'"

L.

, .

::::

It

!...
i

nR,

;
, .
.

11.8.

50 .
,
.

/l00 . :r

20

,
,

.
,

.
.

, .


,
p~a .

(.

22.8),

. -
;


(), ,

Control of Enzyme Activity, 2nd ed. and


1983.
Hers . G. control of glycogen metabolism in the liver,
nu. Rev. Biochem., 1976, 45, 167.
Hers . G., u L. Gluconeogenesis and related aspects of glycolysis. Annu. Rev. Biochem., 1983, 51, 617.
Hers . G., an Schaftingen . Fructose 2-6-bisphosphate two
years after its discovery, Biochem. J., 1982, 106, 1.
u L., n de Werve G. (eds). Short-Term Regulation ofLiver
Metabolism, Elsevier/North Holland, 1981.
Newsholme . ., Crabtree . Flux-generating and regulatory
steps in metabolic control, Trends Biochem. Sci., 1981,6,
53.
Newsholme . ., Start . Regulation in Metabolism. Wiley,
1973.
Storey . . re-evalution of ( Pasteur efect, Mol. Physiol.,
1985, 8, 439.

Cohen

ll,

23

-
.

-,

NAD FAD;


, ,

(
), i .

.

S:l .

()

() , -

-,

ND;
.

().

, ,

z-

.

,

().

, , , ,

,
.

. ~
,

, .

- ()

-,

, -

8 1573

226

..i. KIICJlOTA

23

+ + - -.......~

-- ~ +

A8plI-D
CIIRTAla

IJl- +

88

PI\ +

I1 ,

---._~ 2Pi

IIMp.qtDt8pkTASa

fDrII'I! K~A

. //

()

.............' -


,
. ,

~ + PPj

cn

--
,

--;

()

~-8

(----

)- ()N + - 2-(}- 2-00-

"II" - . + 'II

U:l-,


. u'

.
.
-

,;.

, --
( )
,

'r .
.
}l,I:t - .. 11.

. . ~
..l [ re ;J DHOBb
u.,-'. .

" -

-.

+ "

lI lunO Ll LI

811 l\l J.
.l I\:Ir. J
JtIf -\.w 1. I1

f ASA

."." +

23.1.

:"ILlLI - Ll

,".
lAI'IIIITDlllyPAC

Ll ..

1,

..

..

II'I
Ikt9l3

. +


; ,

-.
,
,

23.1.

23.2.

13-

. ,

-. -

(}/(I/UICHIIt' 1/ f)u'u, J ,)I(lIPllbl.' 1\l1('.Iml1

227

CO-S-

CO-S-+-G-~
-

2-

--s-
-

PIIC.

(2)

13(3),

P-OI", ,

23.2.

--

13-0.

--

-.

-.

-.

--,

~ -
13-0, 2 (. 23.3).

1l0

COz

,
.

- - ,

(.

23.3).

(2 -);

2 .

~-

-,

2()

3(13),

(. .

"-,

20.2).

CoA-.

2 -m.--.
.
F,


.
(. .

123).

3-

NAD 5

3---

13) 7 ( 8)
-, 13-0
(7 5 = 35).
8 -, ,
, 12
.

-.

A1---.

3-

3-

3--;

3--

NAD.

3-

(. .

12

96

228

23

11

- - ---

HS

11

A..3CH2~CH2- - S-

I8" 8" 8)

I88)
-8

~~~;~~~~~88~8rn.~;~~~~---

------------------

------------------8

I'
-

(l

2-

88

'

.!

It

'1

, 2-pl--

- 3 =2- - S-

A,2--

ll+) --8-

R.1b.H.:lCH2-~-S-
NAD+

- 0

NADH

:
--

3" '2
I
I

+ +

- C-t -

S-

88

.? .. 2

SH

-088

11

11

---------- -S-+-,,, S-
-

. 23.3. - . - ,
2-5; -,
( 5). Ll . 5 TCSJ -.

I\' '('1I' 1I U('Ullm ,'71lhl 1'1I(',lOm

129
129

30,5

3935

229

() . ,

/, ,

, ;

9791

40%.

; ,


-
.

,
,

Q ,

2 2 , ,

', .


.
,

, -

(, 20 , 22 );

'

, ,

, ,

(), .

, -

-Q.

(Reye),

, ,

.
-

r .

- -

-
.

, -

, . .

, ;

hia sapida),

.
(-
. ,
-450 (.

(Blig-

--,
-.


6- C IO-

. ~-(" - 2 -,

123),

-;

CoA-

'-
(6) () ,

. -

(- ,

, .

, ,

,
; ,

(. .

, , ,

, -

-.

292).

230

23

1;

C-S-

"~ _

-....
u 8

u 3

8..-

/;

~-S-
6 ~u-66,u-..,n,

1
~
u

6'U (n ) .... 1:J.2.".c


n- 30&pII:

~--'

C-S-

1)

62'TIHC-66~u-..-
(

.... ,... R" puo., 62 '{' .)

!'----

'."""11-0....._
u..

~
~ 11

C-S-

I n-Co. I
. aerporeau .

62.t/Hc.64-u-n

H++NADP~

62'TIHC-64-uc-

64-u-n~
.

"8' -838

NADP+

/)
3

C-S-CQA

6 J.-

---6-=--u-(n----)-....-6--2=-.---...,

!
4

- ..

..--308

In

. 23.4. ... . 64_


u- . 64 -u--. ,
.

O":UC.lelllle II Ulllm .:lI" I(".1m

231

-, ,

J3-0,

-,

,
~~ ,
,

. ,

26- s-

(Zellweger)

CoA-

, J3-0
,

/1 _

u---, /1 4-u---

(.

23.4);

/1-u--

,-qu -+ A2-m---

/1 2 ---.

8
()

de novo

,
.

) ,
,

.
NADPH, , Mn2 + ]
( ~ 2 );

-,

.
J3-0
.

J3-0.

/14_u-

CoA-,

!l (.

23.4),

-- /1 2 -
/14_ u--;
/13--- NADP-

Al-m- 4_quc--

-
,
--

,
-; 2
.

(.

23.5).

. /13_ -+ /1 2 ----

/1-- ;

/1 2 ---,

, - ,

J3-0.

,
, . .
. :

(1)

ND-6

(. 20.4)
(2) -,
-.

, (. .

124).

, (

) - ( ),

--

10-20

Ix

,

,
;

(,

. ,

),

, , ,

, .

, -

, -

232

23
*

- -s----7~'''----~-~.~ -

-- -

23.5.

r:DP+P,

- 2 - -S-

<I.10J1-.

--.

(). ,

4'- (_ .

17.6).

, .

, .

, (. 23.6).

, -

\
\

\
\
\

\
\
\
\

'

SH

4'-

SH

-------------------------r------------------------SH

\
SH
I

4'

'

\
\
\

l1"

88.

"

. .

\
\

\
\

n-

\
\

. 23.6. , Q: . .
' 1 2. 6
(). s-S- . 4'-

, ; .

(Tsukamoto). . ;
, . ( u .
). .

233

OKU('.leHue 11 ,um

n-

HS

~VS-!:)<VS- SH -;::==~=~
Pl8naa ,

HS

-0- -S

.. 8J1 ",

11 '.

Cys-S--

(ltl KOMnneKC)

(~ n '

*'

11

~ -S - - --

()

n () -n-8

-08-1

1
1
_

CHTU8

Cys-SH

~
1111
~-S--2--

NADP+~--------------

D(-) --n-ep

,
"O'l"."

~ I . I
~

.... ys-SH

11

-S-- =CH-

2,-88QI8 ..I 8n-8

NADPH + +-------

-U8

,.

NADP+~----------'

I To8C8p8SI I

Cys- SH

-S-- 2 - -

8 n

""

11

()

-8

. 23.7. II .10. , }' or


2 aroMa. Cys - Il; - 4-.

. 23.6.

(!) (-- ili II lI . !\I

Il, 1 2
'1 . -- Cys.

SH-pynn: 1

: ~]

234
,

6 ,

S-,


().

16-

4'-

. ,-

().

, ,

3-- (

),

".

( ..23.6).

(.

23.8).

-S- (. 23.7).
- (

Cs-' 10-
l2 - ,
.
,
.

) -S-,
4' -,

() R-.

3-

-,

2 ,

(-

);

, .

. 3 ,

3--

-S-.

J3-0;
, , ,

( - )- 3-,
, ,

NADPH,

NADH

L( + )-

S + 7 CHtCO -S -
+14 +14+-

,(&).. + 7.+ 6~O

+ 8Co~ - SH + 14NADp

-
-S- ,

-,

, 15- 16-

-S- .

--Il.

bl

+ .

__~_...;:ill

____

~'

bl

IIIIIIII::;;"-"'_~"'''' ann-<:(

I-<:-

bl

PIIC.

23.8.

'.;} <t.

U"

235

-.

-. --,

-,

2,- -

NADPH. ,

. ,
, -

lQ

-6-

---------::111'--....
------NADP+

-6-

...- - -_ _ _.-..;;_----NADPH
++

3-

ND+~

NADH +

~2

----'

~-

~P

________________________________- J

---:l"~ --..........

' ~r1t-r=::::~;:::::::::::~________~~-____~
8.

lr~por.Ha3I

---------i A&tn-

.C02~

~~ : OKc:ano.tIT
+ .

n nQA

ana

PIIC. 23.9.

NADPH
- ;

a-Keornyapat

"

Jl.
- <l
U-<l- .

236

23

. (, ,

). ,


, NADPH/NADP


NADPH, -


. .

NADPH

(ND-) (. 23.9),

O~H2_0~ _ S - + - 2 _.~ - 5 -

(,

).

-,

,
.

-
-

SH+ 2

.
-u-

:"

?, .
,

NADPH +

-8

NADp

ll
,

'

.....

--

-,--\.. 2 - -S-

--

. - - (.

23.5)

(.

23.9).

ND-

, ,

( ,

NADPH,

A-r =-CH~ - S-

NADH NADP.

2.- -

,
. ,
( )-

NADPH +

2.-

--

(. .

NADP+

13.16).

--

.
.
.

- ,

.
- -,

23.10.

(; ).

"

, -,
, ,

. --


(l')

; -

NADPH-

~1 .

(. ) .
.
.

-,

n- 24- ,
(. 23.10).

237

D. (ed.). he Enzymes, 3rd ed . Vol. 16 of Lipid Enzymology, Academic Press, 1983 ..


Debeer L. J . Mannaerts G. . mitocholldrial and peroxisomal paihways of fatty acid oxidation in rat liver, Diabete
Metab. (Paris), 1983, 9, 134.
Goodridge . G. Fatty'acid synthesis in eukaryotes, Page 143.
In: Biochemistl-Y of Lipids and Membranes, Vance :.,
Vance J. . (eds.), Benjamin/Cummings, 1985.
Gurr . 1. James . 1. Lipid Biochemistry: Introduction,
3rd ed., Wiley, 1980.
Pande S. . Parvin R. Page 143. In: Carnitine Biosynthesis,
tabolism, and Functions, Frenkel R. ., McGarry J. .
(eds.), Academic Press, 1980.
Schu/z . OxidatioIl of fatty acids, Page 116. In: Biochemistry
of Lipids and Membranes, Vance . ., Vance J. . (eds.),
Benjamin/Cummings, 1985.
Singh N.. Waki/ S. J . Stoops J. . ( question of half- or
fullsite reactivity of animal fatty acid synthetase, J. Biol.
., 1984, 259, 3605.
Tsukamoto . et al. architecture of the animal fatty acid
synthetase complex, J. Biol. ., 1983, 258, 15312.
Various authors. Disorders characterized evidence ofabnormallipid metabolism. In: Metaboli~ Basis of Inherited
Disease, 5th ed., Stanbury J. . ! al. (eds.), McGraw-iIl,
1983.

24

.... ll
'J

( 2) ,

.
, .

, ,

24.1

~[,

( .

15).

2 '
C~2 24 ;
-

, u-.

. ,

, .

, ,

, Ol'

, Q-

. , , , ,

JWm.IU IIll"1l1l ::Ullbl J6

16

239

(",,7, 16:1, ~9)


,
9- (. 24.2)

18

(",,9, 18:1, ~9)

, .

12

/1 9 -.

- -

18

* (c.J6, 18:2, ~9,12)

/19_ ,
;

NADH

NADPH.

, , -,

18

15

*- ""3, 18:3, ~9,12,15)

.-

20

* (",,6, 20:4, ~5,8,11,14)

CoA'SH

CTe.poMn~epMeHT

(c.J3, 20:5, ~5,8,11,14,17)


_ 24.1.
. Rit
. . . ;
(J) (. 007 ~ )
II
( ) .1
. J. .

II
~,


I'fl (007).
16 .
(16: 1). .
JJ

Il

(69). fL-l

.
ll .'1.
16 ; ~
, 9, 12 1S,
. it,

n~

, ll

SH

(.

24.4).

/14_, /15_,

/:1_

/19_

(.

15

23),

nn

- +

/19_

. 24.2. 61)- ~.

24

240

bs

().

(6), - () -
;

, . .


. (.

24.3)

(.

24.4).

ro6 .

-,

, , .

(--). >

- (

) . ,

[\9_ ,

(9)

(.

24.3).

6_

m8

~
I
I

24:1

[bl
8

22:3 - - - - -.... 22:4

1+

I~

-----.

22:1
8

18:2 - - - - : : t..
~ 20:2 ---~.~ 20:

20:1
8

lCR 8

I
I

R
18~2

'~

20:2

~
I

.. 18:3

---~.~

20:3

- - - - - 3.~
.

20:4

----tl
...~

22:4

----~~~

---...;.~

20:4

---~
..~

20:5

----tl.~

22:5

---~"22:6

22:5

I'8

I
I
I

a-R

18:3

----~
..~

18,4

. 24.3. 9. (06 (03.


, .1 .I . blbl bl
.l0 9 .
. r .

. 3

(1)9.8 -- .

Il (J

241

12

11

C-S-

.- (~9.12-u.u-)
02+

ND++j

I
2 2+ NAD+

18

~6_
-ecny38

C-S-

7' nn- (~6.9.12-u..n-)

2
(-. NADPH)
14

wt

"

...

..
'--_.;..._.......;.;(3J'I
..._"_._18_)_.....

11
-S-

20

-,-nn- (~8.11.14 --) ~

02 + NADH + H + l

~5_

-:.838

2H~+NAD+
14

20
.

24.4.

11

11

S-

n- (~5,8.", 14-n-)

; .

- ~6_, .


()

, ,


.
,

2- ). .

n,

1928

. , ,

D,

()

l1 S'"'-

ro 9,

lx

(. .

153)

(. ),

, -.

. ,

, .

, , -

242

24

. ,

,
,

20- ~

, .

( )

1-2%

(), () () (.

-
-

,
.

15). , 2-
,
2
(. 25.5); 2'
02

() , 2 02
()

, )

4
JI (. 24.5).
(

, )

-Ix

- ,
24.6).

, ,

(.

, ,

15%

0-

-.

, u-
; 1:.. , ,
-

15).

(. .



,
.

(.

24.7)

--,

02'

, F,
(2 ) (I 2 ).

.
.

(I 2)

,
,


. ,

-,

, , ,

; ,

, .

, ),

20:5003

., "~'.

bl

11.

8 88

. _.

.,
88

~'~

t(

~~-_

bI

88

8bI

. 24.5. . -
. . . ,
(). .
. ro ci

11

8 "

+-2

II", 1

'-

I +2

~COOH

~
__
~-2H
w

8,11,14-

(~-)

"~

poct8

,
~nrF1

<D

~COOH

eI1
t

5,8,11,14-
-

11 8 11

~nrf2
'

~0A2

~eA01'Pt1l'Rt,
_
..
...
~T84
. nTC4

wnna

8

o
38

t +2

-2

.,

::::::::
5,8,11,14, 17-

-.

I 3

~eii~;~"W

()

-2

. ':J

86-

&

.:=g.:.======:;'111

. 24.6. 3 . . - ; I - ; -,
-; I- ; 2- .
,
.

ci -

244

24

~OOH

20
l

_ _
7'r"~COOH

I 2

~~COOH
J

11

6- F l

II" I

Y7'r"~COOH

..

O~

~~~2.

/'r"~

QH

~~
.

,--,-

11

(:H~COOH
C-H~

OH~
8
+

/ 2

~""I"---"'""""'-""

~COOH
\'

1~___~_~2

~OOH

I '

I-l"

(f!8301

~COOH~COO=o~COOH

D2

. 24.7. 2- . -;
; I - '; - .
- --. I 3.

2 2 l

, I 2 ,

., ,

, 2 ,

,

.

( ) (.

24.6).

, ,

. ,

'I1 . ,
,

- )

. ,

(1

, -

'

!.lm60.LU.tt Illl(l(",i".\ :CUPllhlX I.lOn1.

II

I\1G1ll

245

~CO~H


8t

15-

15-

115-.no",,_1~0:!

, . .
-.

~H

, -,

15--

\ 12.

. ,

12-

~COOH
HO--~

12-

, ,

~/COOH

~
~CO~
5-

. (
)

".

~5--"'--"'----838-"

.0

~COOH

HOO--~

~COOH

~~-0--'-~

5, 12 15,

~0

5-

, ).

'

5-.
4,

4 ,

(.

24.8).


.
v8"

0NH,NH\

,Ho~'~l

I
______
~

~----"'---+_.1

ll n

4 . ;
-
.

-I't(.,s I

fl

(-)
4 , 4 4 100--1000

24.8.

r------r:-V ):

r--------~

! L .,( NH2

4
4

5. 1 - ;
2- - 4 -';
3 -
-S-;
4 - -; 5 - 3

- -.

r ------...

I -"..

I HO...iIN~~ )NH 21
I

1
1

----y~
I
10

1
1

L-----~--"1-..

~OOH
-

04

--

4
v

~COOH

~
~1\
-----. . .
r

1
NH 2 '\
1 HO-,f\ 1

s I

'------~_
I

..

~COOH

~
8

246

,
.

4
.

, -,

,

(, ).

Hammarstrom S. Leukotrienes. . Rev. Biochem. 1983,52,


355.
/ R. .. Control of polyunsaturated acids in tissue lipids.
J. m. . Nutr., 1986, 5, 183.

24

Kinsella J. . Food components with potential therapeutic


nefits: The -3 polyunsaturated fatty acids of fish oils,
Food Technology, 1986, 40, 89.
Lewis R. ., Austen . F. The biologicaHy active leukotrienes:
Biosynthesis. metabolism, receptors. functions, and pharmacology, J. Clin. Invest., 1984, 73. 889'Moncada S. (ed.). Prostacyclin, thromboxane and leukotrienes.
Br. Med. Bull., 1983, 39. 209.
Piper . Formation and actions of leukotrienes, Physiol. Rev.,
1984. 64, 744.
Rivers J. . W., Franke/ . L. Essential fatty acid deficiency. .
Med. ., 1981, 37. 59.
Smith W. L., Borgeat . The eicosanoids. In: Biochemistry of
Lipids and Membranes. D. ., J. . (eds).
njamin/Cummings, 1985.

25

~

.


. , r,
,

.
.

10%

5--

, .

, ,
,
,

. ,
(

- ) ,
~

]( , -

ro .
,

-),
,
~ IC.

lC 8

,
(, ,

BTOPblX
ropMOHOB, --

.
,

,
. opraHOB (, , ,
, , , ),

e
.

. ,
,

(.

{>

25.1).

, ,
,
.

, e

, l
,

-,

, :

1)

2)

' ,

3) ,
( ).

sn-

-3-. -

2 -

ADP
~

2 -

2 -

HO-~-H_r;:=======:;-,.~HO-b-H ::.~-;::::;;;;:::::~~=::::;---i.~ ~=O..


2 - Inn8!
H2~ - 0 - 0
H2~ - 0-0
sn-nn-

nn

nn

88

n- (
8.,l.,I 8)

I\..L-_ _ _ _

88-

--J

-n8

NADP+

NADPH + +

11
11
H2~-O-C-A,~~~~~====, H2~-O-C -,
hO-
1-n=

8~8

2- -

1-nn-

.
-
(n88)

H2~-OH

11

_..

n-

( 8
8

----

1-nnn-38-8n

Hi-o-

1-n-

2-8nnn

8""

11
H2~-O-C-A,
R

n-

08nnn-

----

..
1
r:\
H2C-O~

1,2-8nnn

8 (w"~)

8 ()

n
(88)

AP~

I8~n8l

~8
n8

PP j

OJ1
(8n8)

11

"

H2~-O-C -,

H2~-O-C - ,

-f.-O-f -

2:
1.2-8nnn

D-n
(COP-8n) .

CDP-~8nn,"'I-------'3~~8n

11
H2~-O-C- ,

1.

H2~-0-C - ,

2 I . 1
2 -0-0

--

8nnn

n
(88)

lIlIlIJI

11

......
-----_}

(Dtln3~.)

88n8

(-)

"

ADP

2 --

8n8

'1

2 -{!)-" ~

8nn

8n-4~

'1
H 2 f- o- C -

H2~-O-C-A,

R2-f.-o-f -

A2-~-O-~-H

2
8

H2~-O-C - ,

----

----

H2C-O~

DP-8nnn

-f.-or -

R,

- f.-o-f -

2-0-0-u-<V

8nn-4,5-8

25.1.
;

. 1 -- ; 2- r'
. J- - ; _

3 --

mUJ Ull.1.'.'Ill 11 llllJlllnll

, ,

).

l:l--

r Jlll

r,
NADH --
-3- (. 25.1).
. IIJI .

(.

44.5).


(~ )

.

. I

-;

, ,

- .
-
-,

1,2 ().

)- -

),

(~OP

). (
) 1,2-
;

\ )

, l--3-

--(--
)

, .

.
-

1,2-.

, -; .


1,2-
, r

,
.

1,2-

-.

- n

[ (~Ta

s-

r-8).

, ,

----,


().

, .

D- (.

NADPH.

sn-r

D- - - - _....

. JI.

(,
), 1,2- (,

~~

).

()
(
),
D- -
,
(. 25.1).

h-4-
, -4,5-.

~PI

()


( -

25.1)

--, ,
. 25.2.

. -,

25.2.

<fII<f.

lll

250

(-

25

- ,

" I-,

-3-.

2 l-,
2---
(
,
. 25.1), l-, 2 (. 25.3).

2)

.
lC

(.

25.3);

R -

"'

HOOC-R

1-r -

~C-O-IC~12-R2

I 881

2 - 0-0-2 - 2 -

NH2

1-,2-8r3-.8

NADPH.0
2

~
'--

1-r-3-

r~8

r
838

I
r.:\
2 - o-\!.I
,-n,2-r-3-.

R-

'

"

--

c--fP'l
2

1-n, 2-r-

, rlo1

R -
}
2 - - ( 2 1 2 - R2

'

--

I I

~ _ o--t;\
2

- ''

1n. 2-8r-

3-

38

R---

~p ~ - - ---~

~C-O-(CH212-A2

H2~ - -I2 )2 - ~

'Ir881

11 _

(8r)

2
~ 1-,2-8rn

3-8

.1"

~~ - o-ICH2 12- Ra.


"-

R----

88-

!
I
_

- I

h b-0-0
2

1-Jlr-

D-3

11
I
R----

10-11

H2~-D-ICH212-R2

- . --

8 I H2~-0-0

h2b-0-0'

1-

NADPH + + NADP+

~~-D-C_R~2
22
H2f-O-ICH212-R2 ~
-;:;~===i~ "''

, l-r-

"

NADPH

3- (. 25.3).

. -
()
- 1-2--sn--3-.
,

.
Wl.

-
1-. 2-r3-

Ic4tl

--I 1
2
2
1,
1
----

2 - R2

H2~-~

1-. 2-8r-3-

PII('.

25.3.

!l.n

IIPOCl

"J . .;!'\1<l.lnI rr!\1~-lI .. ( ).

mtJ'lllJ U:1Z. 106 uu llInJJ6

251

.:n.~..

~ .

11

, .

11

QCnnasa

2-

11

. ,
1-

I
0-

(.

25.4).

25.5.

,
l1.

,
-

.
(,)

( ),
l-


.
--

. .

H1C-O-~ -,

11

I
Rl-C-O-~-H

H1C- --
8

81
I H20~
Idn8 21
2 -

11

H1C-O-C

:
-,

(). ,

I
--

l-

2 -

--

()

H10lI

, -

. ,

H1C-OH
I

--

1 (. 25.5),
- 3 (
1.2- ).

. D.
,
n .

2 -

II:

---

x8115cTepOII-

2 ~

r88

H1C-OH
I

--

+ '

2 -0-

sn-r -3-8

'J'",

II + XOJ1ECtEpOn

lIsllqJII +lbli

3'"'

.,

XPECTrplllA


. 1,
(,

)-
.

25.4.

(t).

281.

2.

252

25

en

-.

,
JIIO

x .

;r

CDP

25.7.

Il ll.

()

, ,
,
. .

(.

25.6)


-;

3-

NADPH

~,

--,

-.
(N-)

11

+N

-(2 ''4--S-

- (.

-- - -

.~-

25.7).

. "'
D-

. 2+

(.

25.1).


, ,

NADPH+H+

- ~

......

~----.;;....;..---_

NADP+

N +

- r

24- ,
,

( 2 47)
. _D 2-
.
( 2 4s ) ,

-( 2 )12- =- - - 2 -

25.7),

-(2),2-2-2-- - 2 -

(.

NH;

()

(GaICer)

Cer

25.6.

. ---.

(GlcCer). Gal, G1cCer-

-.

, - -

Imll.111.J (J(1.1i'J1l 106 u I? nu6

253

UDOGlc

11 I
-

25.8.

\ J.

UDPGal

~ ~..
-------il..~ ()

~~nn
-

..

" If IfR.'I' . L

(n.)

<l8!i , ft-

5' -u _1 .""
(.

25.8).

"I;'

, , ,

(UDPGlc),

Gal,

UD-

Jl (.

(UDPGal).

, ,

UDPGlc

UDPN-

25.9).

" .

( ...21.3).
UDPGal.

), .

3'--5'- (,
).

, (

) .

. IC-

n-

r ~

/'.

UDP

UDPGal

_"-_....;_""'L'---::I.~ ::.:- \.
(Cer-Glc)

UDP

J..

Ce.--Glc-G.I

Cer-G Ic-Gal-Gal NAc-Gl


I
(G M1 )
NeuAc

UDP-N -nUDP

UDPGal

(an-

.
ann~1 )

Cer-Gl-Gl-GIN

NeuA~

. . . . ._~_~_L'-______

Cer-G,C-Ga,

(G M2)

NeuAc
(G

25.9.

l"IlJ10.

NeuAc -

N-

(nn)

~lw n~1

IC (

r.

UDPGlc

<?

./UI

254

, ;

25



()

(, ,

. pa;J 3 : 1) ,

).

.1

25.1.

r.1J11i"\

. -

lC

yIC

Cer-Gl-GlN-l~Fu
-

--

GI--

-.

IC

Cer-Glc-Gl(Nu}-GlN~Gl ,

GMI

Cer-Glc-Gl-(Nu~lN

GMl

,
, ,

Cer-Glc-Gl-Gl~GlN
+ GM2

Cer-Glc-Gl~Gl

(
);
, -

(--

Cer-Gl~Gl

Cer-Gl~S) "
3-

Cer~Gal

Cer~lc

,
,

lC

Cer~P -

,
";

~
~

, ,

, ;

NeuAc--:-N-1Jl ; r-; Gl-; Gl-; Fu-.

25:'
!l . 2)
, 3) .
paCCeJlHOM ,

,
.
. 25.1.

( ,

),

; ~
.

, ,
.

,
,

.
(Neufeld,

Lim, Shapiro, 1975).



:

1)

2)

3)

, ;

4)

Bell R. .,/mn R. . Enzymes of glycerolipid synthesis in


eukaryotes, Ann. Rev. Biochern., 1980, 49. 459.
. D. (ed.) The Enzymes, 3rd ed. Vol. 16. Lipid Enzymology. Academic Press, 1983.
Brady R. . Sphingolipidoses. Annu. Rev. Biochern . 1978.47.
687.
Hanahan D. J. Platelet-activsting factor. Ann. Rev. Biochern.
1986, 55, 483.
Hawthorne J. N., ns// G. . (ed~. Phospolipids, Elsevier,
1982.
Neufeld . F., Lim . W., Shapiro L. J. Inherited disorders of lysosomal rnetabolism. Ann. Rev. Biochern . 1975, 44. 357.
Various authors: Disorders characterized evidence of abnormal lipid metabolism. In: The Metabolic Basis of Inherited
Disease, 5th ed. Stanbury J. . et al (eds). McGraw-iI1,
1983.
Various authors: Metabolism of triacylglycerols; phospholipid
metabolism; ether-linked glycerolipids; sphingolipids. ln:
Biochemistry of Lipids and Horm&.es. Vance . ., Vance
J. . (eds). Benjamin/Cummings, 1985.

26

, ,
, ~ ,
,

, .

(
);

.

pallero

.
, -

.

) (
) ,

, ,

~.

.JI :

),

, .

5%

()

m ()

, ( ,

).

, .

26.1.

l3

- lI.

JI.


(Sf). Sf 10-13 /

(.

26.2).

lI

NaCJ

1,063)

1 / 260 .

.
.

, -

26.2

26.1.

. bI

r/IOO

570

142

36G-820
8G-180 ')

215

123-390

1)

5G-200
5G-130
15-35

200

107-320

55

26-106

<0.96

()

1.006 - 1.063

( -

< 1.006

(-16 1 )

12

-- I

()

(-

(
) 200 800 Mr/IOO , ~
45% - , 35% -

1.063 - 1.21
()

, 15%- 5%-

11

~I .
;I

.1

26.2.

(4%

25

).

PIIC.

26.1.

."bI
.

!l 1I_1ClbI
,

06-

Sf

r-

(-

0/0

100-1000

<0,96

>400 1-2 9899


2G- 7- 9G400
10 93

88

56

20

15

3G-90

0,96-1,006

25-30

1,006-1,019 1220

11

89

29

26

34

2G-25

1',019-1,063 212

21

79

13

28

48

10

1G-20

J,063-I,125

33

67

16

43

31

10

1,125-1,210
> 1,2810

57
99

43
1

13

46

29

()

()

()

()

?
)

7,5-10

6
100

- , ( ) 'I ,

9 1573

1,21-1,25.

258

)
.

,
,

(.

26.1)

1)

2) R

( --),

;

3)

26

..

60%, 1%.
,

, (

) ,

~.

, .

(.

26.2).

( -),

4)

),

, . '


,

(.

26.2).

()

,
.
(). ,
-I -.
,

(, -)

(, -)

26.2.

_"1 .

6. , .'l08 !ld ~
. .1 .

lIllllm

26.3.

u n/llI .ln

259

D ,:

A-I

28300

( )

B-IOO

17400
350000

200000

-48

,
, ,

-I

C-I

7000
8800

8750

32500
34000

, -

- ,

HI.

, ,

(8-48) ,
(8-100),

. -48

, B-I 00 - . ( ,
, B-IOO, -48.)

C-I, -, C-III

. (.

()

26.3).

, , ,

, , .

. -

5%

A-I

:-

. 81
, 0,1
2 ' -1 .

io

(,

I ,

),

.
(l00/0

, IC

),

. ,

5-10%

.
-

0,5

,- I

I.

0,7---0,8

' - 1. -

260

26

.2 ,- I

, ,

.
,

50%

. ,

25-50%

. -

(.

, , ,

, ,

26.3).

).

( ).

. ,

, -,

; ,

, ,

. ,

. ,

, .

, ,

Z-. ,

, , ,

( ),

Z-

()

-,


,
()

. -,


.
(. 26.4 26.5). ,
,

, -

( ),

-:

..."

'.

"

:~

."

".

:.

0.0

()

...

"

.. ..
R'

. 26.3. () ()
. - '.l ; - ;
- ; -- ~ - oll0; - ; - ;
- . . , ' .

lJj
~---_.......~~...

I). 26.4. ~lLJ . ---- ; - - ; -- ;


- : ; ll --- .11t-1 -; -- .1 l; -- ; -.. -I .

26

. ~
. . .. . .

. ..

------~~...

(, ,

)
Q

. 26.5. ~1 l I- () "J
(). - - : - --,: ; - --- : - - ; .. _ ; -;
lI ; -.
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- : ~.l ."I u .

, , ,

, . .

;
.

.i, ,

JJ.

. (, )

80,

q ; ,

, , ,

, .

, , ,

800/0 ,
200/0 .

mt , JIIII

. -

llnm n .mu'

263

, ,

'(- IDI ).



. lt,

.
,

, 2

(.

(. ).

D' -II
. -.
,

(.

26.6)

24.5).

24.6

't' .

, ,
, .

, .

, -

( 

; IO

00-

..
. # +-~

+
n

. 26.6. ().

.II1: J1.~lf.l; - rlO.ll;

ri.\",-:\~1 ll-ji.\ ;

' ~Il';;

. XO.1CCTCpO.I:

. -- J1 t-I .l0~ -I
.l0 A-I. ll \.1.1:
. .l I1 . .'l .'l<t :i .1
t-I. ~ 1 - . .l. 26.2. <1.'l .'1 \:IIl 11<1

.? XOJlccrcpO.l. lO t-I.

264

l:

26
, 2,5 .
,

, ,

( B-IOO);

{)

n .

90%

, ,

50%

, 50% - .

(
).

,
().

(.

26.6)


.
,
.

, .

, ;

,
-,

(.

50%

26.4).

50%

: -u ( ),

,
, ,
,


. , , -

, . ,

A-I,

B-loo,

(.

26.5).

.

- . , -, ,


, .

B-l

, ,

-48.

-48, B-l,

, , -
( ),


, -,

, <; ,

D-

, .

. .

, -

tlllnm / .nu

265

n (.

, ,

, z

, ,

nn.

();

26.6).

( 2 ) , , ,

1) .

2) .

4)

3)

5)

.
.

,
, ,

B-IOO,

.
,

.
.
,

f- (. .

284).
(Brown, Goldstein, 1983)

,
(J ,

), ,

(<<
).

, KOTOp.,IX

.



(. 26.7)
,
,

, .

,
. .

,
,

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u,

-,

.
.
.

,

.
,

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, ,


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,

.

(. . 25).

.
.
,

,
,

. -

1)

( ,

),

, )

4)

2)

!tI(jtl

266

:!6

k..

- ....

""ll--,,----- -

f--_ _ _ _ _ _
)

"

11-4------1:1---""'::

-
-
-

bl

bI

bl

-~!--,:o"'~~------ --100

~*~I*"'n*

/.

...-----~-

,\

bl

s'\

1.2 .

I:

: ..

.....
.----

- - - -__-...- - -

/\

. 26.7. ] JlllJ1 (\h ')if ()I\(~lt () R()'j"l~ <1 . fihl1LI


II1.1 .lf 111:1"0.108 11 \.' lJ'r~i!(.lt:t. -II~'~ lt KIICJl(.HbI:
l1lfi II.'1: 1 - .II tlI)ii lI: -
; t1--<t.l\. ;
-
/): : ,)--:f\ . <~II Il~ .1t:1 f rt.:. <\If, If'1 II,"} Id JI l .
2.. .

(.

26.7)

I .

, ,

, .

1.

llIlm

267

11 :n:IIU' .lumu)oH

, .

, . r (

, )

, ,

n .

, .

'

2.

Bo~paCTaeT

- . <;>

, )

. )
, , )

,
(,

). x

; ~

. ,

, n

(. .

31

32),

. ,

, ~ ,

, , ,

~
.

(--J}- ),
,

, ,

, , -,

. ,

.u .

. ,

, .

, -,

,
,

yr

.
, ,

. , S--

NADH.

AIIKoron.. AErIlAPOrE KAS.

J--+ "AD+ - - - -....


t~"Jl
CHa- + NADH + +
AqE"..Atr-

268

NADH

26

. .

, ,

[NADH]/[NAD+]

+:t

, ".

, , -,

.
.

(.

26.8)

( ).
-

-.

[NADH]/[NAD+]

[]/[],
,

. ,


.
,
r,

-450-
,

NADPH

02.

. , ,
':f ":
.
, .

- -3-
, . 25.1. -

-3-
, .

.
,

(. .

262).

. ,
, ,

'.

-l-+ "ADP++ 2 z
...rIt

,
.

- --,

--

.
,
, .

,
,

;
,

, ()

(.

26.8).

, .

. .

. -

nu

IUnu8

269

-6-

j "-"' 2

-.

l . ,__

NADPH+
+__
.;a...._

f__

-3-

------------

!j,

puuUR

---

I
IL

__ _

unu

2)

1)

n...,...

/lll1Ot1PO......

..:

(, )

. 26.8. _l . -.'I , .
, t-I. . l .
. -- . 25.1.
- ; --Jl; - ; -
.

,
--, .

-- ,

2 ,

"

,< --).

(.

2 ,

}I ,

26.8,

1),

Q }6

270

. ,

Hd

2);
1

, .

, ,

. l1I
,

COz


'

. .

--

-;

. ,

,
-


, .
fl ,

. ,

in vivo

(.

19).

.
-

, .

. ,
,
.

51-

-3'.

5'-

, , .
, .

.
,
. .


u ,

.
,

.
,
. OC~OBe
,

,
'.

, (.

26.9)

. ,
,

II ,

. .

, ( ), -

f3- ().

(), ()

. ,
.

. r
- . -

Tpallf"l1f1{IIIl Lt II('(IIII((' ,""lItt)(J({

(\J[,)
,
1 ,
;
8

"",-"'\

I3-' tI

11

\
,;.:

,"@ \

271

j(t)
t

:>

t:::\

'Ja'---~-"'"

GTP

./

,---------------------------,\
opmoh-8

()

"
poca"Je
'

....:

././

/'

"...

~ -

8tf

,/

8
(, )----

."

....... '0

Al+
n--n8

. 26.9. .1 .l.1 , TI" "- lii 10PMOII;


,,'80;1 .
JIII'\ , .: i:l:,t;lJ .I.m:I }f IIJll~ :\ Jl 113
\;l,. .1'J (()~l : 1, ;li.\:I :) .".I: 2) ,l,n" .1; 3)
l If .1i.ll1: \III KOHI1C~" I ; 4/ /')8.II ;I.r 1~l2\ :\1:
5) . Ii.t1I1 :IJIi.l. '~, . Tl' 11 '-.li1I Rl ;l.'
lI iJ1 vivo, I. 6.1:\1 in "jtro l1 I " I':VR ' IIJ\:.
!\1 IR. Jl\.f .lII~1 i<1J1 II<.bl:II ( + ) rIl<l rC_lbl.IL' ( ) I.1II :~.

:IIIII'"1II '.1

J\) "rR.

. ,


),

, .

, ,

. , -,

-; ,

.

6- . -

, , .

272

26

. -,

""

"

.
, ,

--------

I
I

, , ,

, r

..

"


" t.

CJ~lll T~M~~~


(
), () (
, ,

\;!;I

.'+'/~~=======~~

), .

-,

; ,

, .

'

, ,
,

, .
,

/j-8i

, -
.

r ,

,
.

,
,

-,

. ,

, ,

, ;
-

(.

26.10).

. 26.10. . "
lI
. 80
II .\1 .1.
. lI (K.iK ')10

F ,-), )l
. ;n, .i TKlHb
, + 110 I " l
t8 .1t. ')10 It
maCl- l.1. !'\1 .i
(') Il.1-.
:\fI -,
!l1 l! . .1
inI

(f -

)n

.'L ()
} J.

ll ullu uu

Brown . S., Goldstein J. L. L.ipoprotein metabolism in the


crophage: Implications for cholesterol deposition in atherosclerosis, . Rev. Biochem.; 1983, 52, 223.
Cryer . Tissue lipoprotein lipase activity and its action in lipoprotein metabolism, Int. J. Biochem., 1981, 13, 525.
Eisenberg S. Lipoproteins and Iipoprotein metabolism, Klin.
Wochenschr., 1983, 61, 119.
Fain J. N. Hormonal regulation of lipid mobilization from adipose tissue. Page 119 in: Biochemical Actions of Hormones. Vol. 7. Litwack G. (ed.), Academic Press, 1980.
Fielding . J .. Fielding . . Metabolism of cholesterol and lipo-

273

proteins. Page 404 in: Biochemistry of Lipids and Membranes; Vance . ., J. . (ed.), Benjamin/Cummings,
1985.
His-Hagen J. Brown adipose tissue metabolism and thermogenesis, . Rev. Nutr., 1985, 5, 69.
Krauss R. . Regulation of high density lipoprotein level, Med.
Clin. North ., 1982, 66, 403 ...
Lieber . S. ll and the liver: Metabolism of ethanol,
tabolic effects and pathogenesis of .injury, Acta. Med.
Scand. (Suppl), 1985, 703, 11.
.
Sparks J. D., Sparks . . Apolipoprotein and lipoprotein
metabolism, Adv. Lipid Res., 1985, 21, 1.

27

,
.

500

CYT- 1),

(- 50%

),

(-- 15%)

( ).


.
,

, ,

. .


, ,
.
(
) .

, -.

.
, l


.
,

.
.
.

,
.
.

/ .

,
,


(.

27.1, 27.2

27.3).

1. ,
6 ,
(. 27.1). 2.

:! ~
(.

27.2). 3.

4.

, .

5.

. (.

1.

27.3).

(3-

-3--)
~

(.

28).


-.

,
,

(. .

28), ,

lllllnt' . PlJIIC110"", :l'U XO.Il'cepo.1O


r -

011

] -

- S -

2- -

II ~

_ 2 -O~ _

CoASH

.
:

::tll-:~_~ _s -
.

, mllII
. '

011

- - 2 - -' 2 - - S -
I

3--3-- ( -)

4. ,
27.3).

(.

SH

2,3-0 Jl

, (i

-- 2 - - 2 - 2-
I

. ,
lt--Jl-,

01.

(20%)

'-CoA-PEYA*~OPH+ 2

-
(
mPllhc--
-).
-.

2NADP ~

Jl. ,

NADPH

CoASH

01.

27.2),

11

r (.

S-

,.....-___- - - - - I --!

275

14

13'

CI\--Ha '4'

\IIn.10Ilt.
- 3-3- 1. --J13 I-
1Jl, tIC .13 l1 .1, 1 -.

27.1.

5.

(.

27.3)

Jl

C I4

2 ,

14-

.
- (

4 ,
:.

, --,

Cg

- -

L\7.24-.

-.

-- ).


-~. :,
-
(. 27.1).

NADPH,

'}

Cs

Cs

6 ,

. ,

. ,

2.

(.

27.2).

-5-ll .

3.


HeM .
(
) ll.

.
,

,
. , ~
. -

':"

'/

"

/
CH l

"

27

"

,/

L--_'-_____....

,/'-/"
2

CH l

8-5-8

- ~92.TP

'---.....:'-='~.:....:.:..:'-":..:.::....::=~......

,/
/

0-

~.

-@-@

, , /

"

,- - - - /

"

2
/,

2 + ;

-- - 2

.-

- / f '

"

"

--

-5-

-nr.... ~H. ''~ fH.

lo'

I I

--5-

,.)

-~gl/

/ "
2

0-

__
-

,
I
I

2
"

0- -

"'

II~NADPH+H+
M g 2 '
2',

2,

NADP+

27.2.

, . ----'''Jl.

. . . '

C II

C I2

. . r- !\1 . ,- ll .

, .l

277

)
.

11

) - - 5 - - - . - - 2

-7 -

01.

[.

01.

2 - 2 ---'OC,:----~,~--t~ ) - = - 2 -

]
- -....

,!
NADPH

02

02, NADPH

NAD+

14-
2~

22

!~ I

.......0""'0

18
/0

(:
2

120

.....

' 12~/17'n
111 131 D 181

23

28

25 .......
24._0
'.27

NADPH

;!I;::::;:=====:;-_
~1~~D~ 24_
19

110

81
7

~,4/'~8/

'

(24- )
47,24 -

. 27.3. . . .
. lI . 27.2.

278

17

--I).

--

()

ll

16

3-7

.. n

(1

. CoA- ,
.

CoA- ,
.

-,

CQA-I\,

(.

0J1

CoA-

27.4).

0,05%

70--80%

, ,

, ( ,

) :

. -

de novo,

2%

(R)

1
1

-.=~:~

10

(88R)

I
I
1
1
I

<t)1I

8''"
-1(!)

~-=~====~~/@

.9

27.4.

(88R)

- - - - -

I,

J "---.....,.----...

I
1
1

I
I

I
I

_______ ----.1

&il!\1 .rIJ IC"Ja XO:ICClepO.'1a I\I-<":-&1.

Ull. mllnm u :llU .1m

279

ZRR nn

8R

./

/'"

'" ,..-------'"I

I
1

r-----".<!)

I
I

----------~~--~

'
EXATI

~--------~----~
I

--------------------,

L__

- - - - - - - - - - - - - - - - - - - - - - -

I~

. 27.5. . . .- 1Jl K:ICT()'1HOM . -

Jl; - - r.l;

;-:L'.'1 ..r.
II:.l.l 1: -I
l
- 1I.10. Il
.III Jl .

A_I.

3) .

. -,

, >.

4) ; 5)
, 8

(. .

.18.

264)

1)

, ,

. ,

. ~

2)

, (-:
).

3)

~,

( -8-100, )

, .

.

1)

27.5.

--I .

'J. ;

--

2)

de novo

280

27
.

, -,

,
.

27.5).

(.

--I00,

(-).

, I-

, ,

Pf
(.

27.6)

/100 ;

, .

. -

200

WQ-Q

I J'
(0,5 /) 8
...

(0,5

/)

....... -..

. 27.6. 8 .- . - ; ' - .
; - ; -- :

.l0;
- r
8
;
--:;
-:.'18I1:
A-I-- ..l A-I; --
;

- ;l.

ll.

mpollcnopm u JljU 10

281

, ,

.
, ,
(
) (. 26.6).

, .

80-900/0
j

().

II ,

50/0

.
-
.

, , -

Ix

.
, ,

. 6 ,
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,
.


.
.

, ,

; ,

,
.


~. (

yr

.
, ,

,

.)
, --I.

, .


(.

27.6).

IU- II.
.

, 2
(.

26.6).

. . .

, (.

27.7).

, ~

-, -D.

,
, , .
, -

7-

( ), ,

.
7- -

282

27

7-

C-~-CH1CH1-S0)H

11

O~

CoA'SH

11

~&9 C-S-

11

( )

CoA~

.7

( )

luu
+ 7-zu

C-N-CH1
11

uu8aJ

'

( )
zuu

+ 7-luuuu

( )
PIIC.

27.7.

8
( )

J 11 ll.1 i.'I' I.I. J;\'I<l:\. II. <t<t:\.fh1 .. I'I


l8.

NADPH

-450.

7-

,
~ .

, - -

3 7 .

7- .

, . .

- - (.

27.7).

CoA-

--

12. -

CoA-

(.

27.7),

. .

lim(' J. mltllm JKC"pt:l/lI.<1 XO.1t:('m('pO,1Q

283

3: 1.

--,

, 7a-.

: ,

. ,

7
. 7

7-,

: -

).

(.

27.7).

-
;

7- --

,
,

7-. ,

100 ),

98-99%

r ; ,

--
.

, ,

. ,

. ,

500 jCYT,
.

. .

(Questran).

.
( 3-

),

6--10

, . .

1-2%

, ,

de novo

,
;

.
.

,

.

-
- ,

,
,

7-,

, .

~ ,

(.

- -

27.1).
,

. , -

284

27

1)

2)

. ,

.
; 3)

; ,

( 2 )

'

, -

l\

, lI,

--I. ,

, (,
, .

, io

),

. ,

. , ,

, ,

, ,

, ,

,
, ,


. ,

. , , eM~
, ,

l:l.

8e ,

. ,

. , , , ,

. n

, ,

, ,
,
u, .

.
CoA-- ,
,
,
.

- .

(Questran)

HecKO~KO ,

umn, mllnm m.f

285


, ,

. ,

.
-13-, ,

13-,

-13-

, .

-,

- .


()

--II,

1.

( 1).

- .

,'

, ,

. ,

, ,


- 11. .

2.

11).

13- (),

(. .

26.4, 26.5

27.6).

11
.

1.

13 () .

(, ,

, ,

). l Ia

11

, -,

- lllUl
";
.

.
-.

2.

50%

00.

(
)

. -

, ,

Hy .

3.

ll1l, 111 (<<

-,

3.

-..1

( l').

).

. -

lll

286

1,019.

27

6.

r.

(-).

. , ,

. .
. ,

. :
()

a .

n -
(

. .

: 2, 3 4).

. ),

III

-2.

-.
4. r- (

IV).

().

- .
(-),

'I

....

ll .

( ). ,

~ -
13- (13).

"I'

,
.

11,

~,

1V

, :

.
.

5.

ll \.


X(lp(lKTep~

, ,

,
H~pM. ,
. (l

caxapH~ .
J ... .

.

-,: .

r'n .

S., Goldstein J. L. Lipoprotein metabolism in the


crophage: Implications for cholesterol deposition in atherosclcrosis. Annu. Rcv. Biochem., 1983.52, 223.
nn, '. ., nnOl' S.l.. The dietary treatment of hyperlipidemia. Med. Clin. North ., 1982, 66, 485.
Eisenbcrg S. Lipoproteins and lipoprotein metabolism, Klin.
Wochcnschr., 1983. 61. 119.
F R., Sobi1le J. R. (eds) Cholestcrol 7 a-Hydroxylasc (7
Monooxygenasc). CRC Press, 1986.
Pielding . J., Fielding . . Metabolism of cholesterol and lipoproteins. Page 404 in: Biochemistry of Lipids and
nes. Vance . ., J. . (eds) Benjamin/Cummings.
1985.
Pmchlich J .. McLeod R .. . 'Lecilhin:cholesterol acyltransferase (LCAT). Clin. Bioche. 1982, '15. 269.
J. ., "l R. J. Treatment of 11ypercholesterolemia,
. Rev. Med .. 1986. 37, 427
R. .,lmll"llr;I.~ . L. Li
.~"
~tors :!d cholestcrol hstsis, 13iochiJ
;: ~ 1.:" . Acta., 1983, 737,
197.

28


()

("" 40%

).

', ,

. ,

(. -)
; - .
, 8,

. ,

?.

, .

(, ),

. ,

).

,
n<

, .

26.

(,

),

(: 28~.1).

(,

) -

(,

0,8

0,3-

/ ,

).

n , .

10%,

288

28

. , -

10

. ~ -

de novo.

-
,
.

0,2

1,0

0,5

2,0

3,0

 (l - 1 )

. 28.1.
.

3 3 2 .


. -. . (
D.L. Topping .)

-, .

-
-D-
,

'

[TP)/[ADP),

,
,

(.

21.2).


, ,

, ~

NADPH,

jJ ND-

.

[NADH]f[NAD+]

[-]j[]

(,
)
,
, -3-,

22.3). ,

(.

, ,

.

(
). ,
--, ,
.

--60
, ,

-,
.

. --,
- (.

CoA- , ,

cAMP-
--
.
,

23.5).

- -

-,

/ln

289

CoA-
2 + /
.

11
-)

.
,
.

11
-- 2 --

888

D (-) -3-
,

NADH + +

--
;

t1 -- 1 --

D(-) -3-v

28.2.

(-)-3-

, .

, M

()

.
-

,
,

( - )-3- (~-
), <n .

.

. -

),

( ),
1 (.

28.2).

. .

D( -

)--

[NAD+]j[NADH]

r-r

[-]/[

1:1

10:1.

1 MrflOO

( ).

.
,

, .

,
, ,
.


.

, ,

. -

3- ,
. ,

, .

\0- 1573

-,

.
,
,

.1

290

-+----

...

~)

"\

II
\

"
\

... -...., '\


/'

\.1

ro
1"

l.I.-

1"-

1"

Ke1"OHOBble 1"

\,

------------,I
\

1"-

- - - - -

1
,

/~/

,-

28

,,

"

.... ?"'-_ ...

28.3.

. . .




, .

3--3---
. ,

. 3-ll-3-.lJ
--, - -.

(.

28.3).

- (.

I1YTL

, OTBeTCBe~He
, .

,
aT~MOB

. , mm

,

,
,

, -,
, .
jJ:
,

28.5).

-.

, -,
,

OllSl (


).

.

--.

--.
:, ~
.

( - )-3

D( - )-3-

D( -

)--

. J3-0,
- (.

28.4).

-.
--
28.5)-

-3--

(.

(-),

3-

-.

n ll

291

- -.

88

1-

COA---.

+ + CoASH - - - -......

2 -

-.

2 S
Auur -

-,-
-

D( -

+ :.

)--

;
'

2 -

~CH2CO.S.COA

8u

u-

uu-

)--~

D( -

NAD +,

-.

I -8 I
2 S

-, ~
, .

-;

2 -

(. 28.4).

2-

ATP

--

.----t-)

epuu.IJR
--------,l~~,

Il
I

,"

~-----------------~~--

1 '--~
"

",
2
\

(
,,
I

~----

l"08

,I
_'

D(-)--l"- ~NADH + +

D(-) --l''l''

. 28.4. 3 _ - : ----.

292

11

11

--,--S -

28
r r

.11

]- C-S-

I I

(1)

in vivo

CoASH

,
.

1
]- -

11
C-S-

. .

300/0 ,.

,
,
(. 28.6). (2)

~------)

11

11

1 -

- C-S-

--,

2 -

IJ-o
-_

. 28.5. -
( -).

70

, .

_
,

(3)

/100

, -,
, ,

!
!

_ K~

. '

r-I----.----

, _---
(-)

. , ),
,

~~ puuUUR
~

o~ n n..
"",,
0

KeroHoBbIe ,"


, .

D( -

)-3-

in vivo

28.6.

. 1--

(),

.lJlU .m.l.\t{l unu

u LtmlIuu JlluLt

293

--

r r-3-.

rr

3-

r-

r r

3-
;
, in vivo
.

- r

0 2 i

r-

,
I
r (.
r

25.1).

02 , r,

, .
1 -
r 02
r 129 (. r. 23);
,

33

, 3-rll

r r

14 -

r (

, r ,

r ).


14 0 2 14

21

. , r

r r

r.

, -

1,

(.

23.1);

[NADH]/[NAD+]

. , , 3-

, r,

r, r ,

(McGarry, Foster, 1980) , - -


(. . 23.5)

ter,

) r -

-. r

,
r .

(Ut-

) , ,

~-~. . ,
r
r -, r
r - 1 (.
28.7).

nrnox yr,

"{

.
r,

r (.

r,

- ,
-

-. ,

-
.

8JI , lC

28.6 28.7). (1)

,
.
r
;

, ,

. r

[J/[
] , -

r.

(2)

294

28

..................

"

..

"

....

....

~~~~K~:

--.... --

.......------:-~~

J '

"I~

3uUIIUR

_.------I.~l

;~v

ro

~"

I~

.!K~:n'~.

.. .0

28.7.

ru u . -- ,

. ()

() r , -

. - 1,
()

28.8).

. ~

(3)

~ (.

2 ;

, ,

in vivo

ll -

,.

. , -,
,

,
. , .,

( );

. , ,
,
. -

(~
).


Ix (:
(. 28.9)
, ,

lln

295

RJI

-,

x (

-)

. . , ,

, tl
,

-,


, -

~ -

_m,. ()

..
~

0"",,-_--

..

.... ~ 't

.',

~1

.
..

.- ~

. _

.....

~~
"

~:
;.

lTr

,S~~~~ ()38

_ n""'-r ~

~
t--------------_....: '
......

....

1'"

. 28.8. , . .
-- k3 ; -

; - .

296

28

...

( ~::~:)

__1--_

11 /k.

+--

8(

:.

--

ce~~

l ~

l.....-.

/i

...8(f-----i~~

anl

j[8

....

PI

----,!I
.~
.

epl

308

~":"'nY""f

- -------i.~ -

/\
t

1"

28.9.

{"

1"

, ,

-.

( ) ,

, .

.

.

, ,

.
,

-,

,w.lu.' n

297

, ,

- --

(. . 23).

Q. ~,

. ,

),

(2)

(1) (,

(.

JO.

(3)

28.8).

(
,

),


.

. (

),
, (. ).

,
- lC.

,
,
, ,
.

, ~,

. ,

..

, ,

-3- ,

,
, x .

i .

()

.
.

.
,
, .

( -3-)
(

).

(
)

; (

) ,

--, ~

, (0'7----0'8
- I 60--70 Mr/lOO ).

mo

. ,

298

28

-,

(
, )

. 28.8. ,
,

. , ,

, ,

, , .

' .

..
500/0
lt .

, 6u

Cohen . Control Enzyme Activity, 2nd ed. m and


, 1983.
,
HeuL., Vande WerveG. (). Short-Term RegulationoCLiver
Metabolism. ElsevieriNorth HoHand, 1981.
'
Laker . ., Mayes . . Investigation into the direct efects of
insulin hepatic ketohencsis, lipoprotein secretion and
pyruvate dehydrogenase activity, Biochim. Biophys. Acta,
1984, 795, 4.
Mayes . ., Laker . . Regulation oCketogenesis in the liver,
Biochem. Soc. Trans., 1981, 9, 339.
McGarry J. D., Foster D. W. Regulation hepatic acid
oxidation and ketone body production, . Rev. Biocm., 1980, 49, 395.
Siess . ., Kientsch-Engel R.l., Wieland . . Concentration
free oxaloacetate in { mitohondrial compartment of
isolated liver eells. Biochem. J. 1984, 218, 171.
Wakil S. J., Stoops J. ., Joshi V. . Fatty acid synthesis and its
regulation, . Rev. Biochem., 1983. 52, ,537.
Zorzano . ' 01. Efects of starvation and exercise
trations citrate, hexose phosphates and glycogen in skeletal muscle and heart: Evidence for selective operation of
the glucose-Catty acid cycle, Biochem. J., 1985, 232, 585.

111

29

29.2.

,
,

(.

29.1).

Arg 7 ( Glu)
His 6
Thr 6
Met 5
Lys 8

20 .

. , ,

(,

Va} 1
Leu 3
Phe 10

Trp 5

29.1.

Ala 1
Asp 1
Asn 1 ( Asp)
Glu 1
Gln 1 ( Glu)
Pro 3 ( Glu)
Ser 3
Gly 1 ( Ser)
Cys 2 ( Ser + S>-)
Tyr 1 ( )
l 1 ( Lys)
} ( Pro)

59

r11

17

11

21

21

, ,
,

, ,


1)

,
.

!)

. ,

, ,
x (.

29.2). , -

300

YTp:lTa

<

29


12 (. 29.1)
,
(Cys, Tyr l)-



, -

.

-
.

.
,

, ~
,
(, ) ,
JI


() .
,

29.1).

(.

L-
, -,

(.

, -

-o~o

29.2).

NH+

-o~o

L-

NAH(P)H+H+
.

29.1.

NAD(P)+

, . -
NH~ NAD(P)H.

NH+
3

:~~o

NH+
3

H2~~O

L-

L-

Mg-ATP
.

29.2.

Mg-D+j

, .

Ullll lOU !10

NH +
/-

11
/-

>-<

.
i.l.
i.l.

Glu Asp
-
29.3.

Pj

301

D-3- (.

29.5).
NAD+

,
, .

, .

~ ,

- .

NAD(P)H,

(
).

.

, ~

L-

, L--

(.

(. 29.3). -

(.

29.6)

29.7).

.
-

I:'

(.

acnapTaTa,
. (. 29.4),
(. 29.2).

29.8).

, , ,

, '

3-, 4-,

. ,
PPj , PPj

'-0<"

NHi

'-"

L-

m-4-.

-0-

"

NH

NH+

.~a

L-

Mg-ATP

Mg-.+j

. 29.4. , . ,
(. 29.2). (R"7'NHj)
.

302

29

'..

~o-

0-0

~-

KNAD+

NADH+H+

:~

~o-

0-0

) 1""'________.3I_

r:-

.....

AK

N +

~o-

- L -

f:H'O
Pj

N +

r'v 0

L-

29.5.

~+/~

. - - -,
- -.

H/t')('0-

, ,
,

,
,

1. . ----,

),)

. ,

29.6.

0')('"

(}ll (}Ll

- 4

4 ""

( )-

~o

303

, ,
).
,

(. _
.

29.7.

. . 4
.

(, , ,

55).



, ,

Fe+ 2

, .
,

; ,
.

--

02

-- (.

29.9).

( )
-

~oifol-

/VV'

Pro

[ 180 tc

/"V"'V'

V"'-A.

NADH+H+

HzO

{ : NAD+

~O
~H ~H+
l

- -,),-n

~H'O

, -- ( -

182).

~~H+

6.2- "-5-

S-

29.10.

~NAD+

~H2'

'

l-

s-

(. ."I).

~NADH+H+

~o

29.8.

. 29.9. , -.
.

~O

18

,"
,

Pro.../'V"..
I

29.11),

(.

, -- ( .i10,


). , II

,
. -

304

29
NADPH +

NADP +

,--,

2-;-

OCH2-~H-COOH

NH 2

NH 2

l-

L-

. 29.11.
,

.
. 11
NADPH, 1 02 2
.
, . 31.

L -

_ 29.10.
.
, - .

:l

- ,
, (.

NADPH,

29.11).

.
.

, ,

S-

---

N-

. N-

(n,

--,

. .

. ,
.

(. .

(.

29.9).

10.6).

10.

UUllm .UIIOl.10m

305

--

31.

. ,

-
.

.;.

,
.

S-
-I- (PRPP),

N' -(S-)-.

, ,


-
,

,
.

Burnstein

,
,
,

Q- (. .

31).

biosynthesis of collagen, Annu. Rev. Bioc1974,43, 567.


.
Calvo J. ., Fink G. R. Regulation of biosynthetic pathways in
bacteria, Annu. Rev. Biochem., 1971, 40, 943.
Cardinale G. J., Udenfriend S. Prolyl hydroxylase, Adv. Ensi)., 1974, 41, 245.
Rosenberg L. ., Scriver . R. Disorders of amino acid metabolism, . ter 11. In: Metabolic Control and Disease,
Bondy . ., Rosenberg L. . (eds), Saundcrs, 1980.
Tyler . Regulation of the assimilation of nitrogen compounds,
Annu. Rev. Biochem., 1978, 47, 1127.
Umbarger . . Amino acid biosynthesis and its regulation,
nu. Rev. Biochem., 1978, 47, 533.
.,

30

, ,

plAlfl ( 8t8

nOloll "
8

.,. .

838
81:!lTKII)

(20-35 r

(15-28 r

838

Cf~)

I .,' 1

.
. ,

88

(5-7 r


( )
.

. ,

,
.

30.1.

838

8 !lIC ,

30-60

;
Jl.


.
.i'I ,

, . .

. ,


1-2%

x .

75-80%

, , ()

, ', ,

(.

16%

30.1).
30-40 .

5-7 .

),

i'I

: ;

60 il1.\1

307

, .


JI:

, .
.
(,

);

L-

"


,
,

, ,

..'

.
()

. 30.2. .
,

( )

JllleIIII.

. ,
. - ,

),

(.

IC

100

(. .

24),

(),

mt
. -
(.

30.3).

~>

11
2--2

4
1) , 2)
, 3) 4) :
. . 30.2

,
. ,
.

308

NH+

3()

....... , ....... 0R

....... , ....... 0-

L-

11

11

. ,

11

( )

.
,

-.

),
.

30.3.

. 1.

u-.
-- -
, .

-- (. .

31.3).


(. ).

. .


,
.


, .

- -

( -

).

) (.

30.4).

- .


, ,
,

. , L-

, -

L--

L-

L-

.
, '

L-

D-

, . .

FAD

FMN

(
)

( 2 2 )

(.

30.5).

2 2 02 2 ,
(
).

( 2 2 )

9 . ,
,
-
.

L-
-

,
(.

30.5),

,
-.

-
L-

30.4.

- ()
- ()_

L-,

FMN-

,LU.'W UlU,lO

NAO(P)H + +

NAO(P)+
838

309

'/

> <E

0-

11

L-

30.6.

NADP+,

'

NH

NAD(P)+

L-

NAD+,

. .

. . ,?
.

a-8

(), ,
-

. 30.5. ,
L- (L--:

-)_

, , L-

---

.

.

D-

F D-

,
,

D-

D-

(. .

29).

, ,

,
, ,

- .
(


),
,
, ('

),

. ,

.
,

. ,

L-rr

- (.
l

30.2).

-
,

L-

(. 30.6).
;
,

, ,
.

GTP

NADH,

' .

NAD+, NADP+.

, , .
, -.

.
, ,
.


( )

.

.

, .
, -

NH~.

l ()

310

(
)

~O"" / 2 .......

11

- ,

NH+
1
/, /0-

11

L-

Mg-ADP
+Pj

.
,
,

.

-

.
.
,

L-

, .

(.

30.7).

30.8.

, i

r .

.,-6

30.8)-

(.

(10--20 /l00
);

, ,

8 (. .

Pj

29).

(.

30.7).

, ,

H:20~ I I
1

'"""'"
11

2,::"

30.9);

6- 6- (. . 17).

NH+
-

(.

NH:

. ,

'/.

0-

11

L-

. 30.7. . (] Jl:-.1<.
'( "
NH t.J' rh.
ii\-1 . -.

, ,

L-

, ,

.
-

mV.. \fIOI.lOm

Glu+

311

II-

II

500/0 -,

8tt

.
-

Glu


, .

. 30.9. ,
.

. ,

Glu-

( ) JI


, . . ,

~ ,

, , .

.
lC

-.

, ,

, 2 (. .

17).

2 ,
,

, ,

! ( -).

.
(

!(

, )

, ,

(,

) ,

30.10

50% ,

. , ,

312

.u

30

(}N

\ \

~\r

V.I

/)'"
Ala

1/
/

Gln

",

Ala

Ala
\

~ ;~::,:

. 30.10. Q nOCJlc
. J10 .
( Felig .: Amino acid metabolism in . nn. Rev. iochem.
1975:44:937.
1975 Annual Reviews. Inc.)

, .
-


, .
~ ,

.nI<038

"

lI

, . .

~-:;

(.

30.11).

..

. .

2~ 30 .

-,

. 30.11. - .
.

1 ,

, ,
,

. ( Felig .:
Amino acid metabolism in . nn. Rev. BilJcl,('m.
1975:44:938. {") 1975 1 Reviews. l.)

20%

(.

30.12).

) .

60%

), ,

. ~

50%

2-3

lIllU.'lU/ .'U/lO

313

Q~

Al,

(60%

~---- Ala------~

30.12. .

90--100%.

(2

j,

, .

J.
, 1-5 .

30.13.

, (N-)

. ,

6 ,

. -, ,

. ,

(, )

, ~
. ,

300

100 I

100

95%
5% -

5.

16.5

2,

, . .

- .

. 2 , .

1:

(l ),

, ,

80-90%

. .

Mg 2+

. :

30.13;

u-

Mg2+.

, N-. -

ll

114

3()

.--_ _ _--,* =

2Mg-ATP

>

N-

. 2
\.

H~O

~2N+"----I~1 ~H2-~

G)

2
I

2Mg-D+ j

2
I

I
H--N +
I
-

H--N +
I
L-()

L-

-11
--

r---------~.

$...
( I

'-.1 2

=
I

2
I

CHrNH
I

2
I

H--N +

L-

H2H~~-H
2

L-

30.13.

II!\1 . .1 .
ll 'I. LI '\.1 !\1l,hl .

2: .

Pj

L-

. ,

4:

llllll

.

-

.
-.
J

,
;
.

. ,

5:

-, -

. -

3:

315

umV.t lImu 1I.i1lm.lOm

2.

, YJO
.

, ,
, .

2+
2 +

,
(

30.13).

1,

11

',

2,

30.13).

X-.


r rr

(, ,

, . .

30.2)

. ,

-
.

, .

-,

(.

30.8),

, ,

, ,
.

(1-2

f'Cyt- 1);

ro

(.

30.13).

( 3, . 30.13).
.

-

25

. ,

, ,

5).

(
(

1),
2)

( , . )

, ,

1 2,

. ,

, (

( ) (. .

32.2).

), ,
, ,
.

, ,

316

4,

30.13).

3(}
.
.

.

, , .
:

,
. ,
,
,

,
.
.
,

.
',

( ). ,

5,

30.13)

-. ,
,

, , , -

Adams ., Frank L. Metabolism ofproline and the hydroxyprolines. Annu. Rev. Biochem., 1980, 49, 1005.
Batshaw . L. et / Treatment of inbom rs of urea synthesis.
Activation of alternative pathways of waste nitrogen synthesis and expression. N. Engl. J. Med . 1982.306. 1387.
Pelig . Amino acid metabolism in , . Rev. Biochem.,
1975, 44. 933.
Msall . ' /. Neurologic outcome in children with inborn
rors of synthesis. Outcome of urea-cycle enzymopathies. N. Engl. J. Med., 1984, 310, 1500.
Nyhan W. L. Heritable Disorders of Amino Acid Metabolism.
Patterns ofClinical Expression and Genetic Variation, Wiley, 1974.
Ratner S. Enzymes of arginine and urea synthesis, Adv. Enzymol., 197 , 39, 1.
Ratner S. long view of nitrogen metabolism. . Rev.
Biochem.. 1977, 46, 1.
Rosenberg L. ., Scriver . R. Disorders of amino acid metabolism. Chapter 11. In: Metabolic Control and Disease.
dy . ., Rosenberg L. . (eds). Saunders, 1980.
Stanbury J. . ' /. The Metaboli Basis of Inherited Disease,
5th ed., Gw-iII. 1983.
Torchinsky . . Transamination: Its discovery, biological and
clinical aspects (1937-1987), Irends Biochem. Sci., 1987,
12. 115.
/ . Regulation of the assimilation of nitrogen compounds,
. Rev. Biochem., 1978, 47, 1127.
Wellner D.. Weister . survey of inborn errors of amino acid
metabolism and transport in , . Rev. Biochem.,
1981, 50, 911.

31

"

L-
,

,
.

TpexMepHYI9

(. .

()


.
,
.

,
, - .


; ~

.
,

( )

6 7).

,
,
,

, ,

( Vm
)

. ,
,

(.

30.13),

.
,


.

.
_

(,

Stanbury et al., 1983).

L-
.
.

. ,
,


L--

(<< ).

1920-1940

. ,

1940

318

1950

.),

31

31.1.

,
(13

Jl ,

), ( ),
,

(5

) (.

31.1).

L-a-

rr (rr
)

(KeTOreHHwe

rr

crr
KeTOreHwe

, ,

, ,
.

Ala
Arg
Asp
Cys
Glu
Gly
His

. ,

31.1.

, ,

Leu

Met

Lys

Ser

Tyr

Val

-. ( ;
, ,

) .

(, ) ,

(,

).

(. .

30).

24),

. , -

~"rg

~
Ile
':~

(. .

~---------iUGI
4

~~

r
/

'"

v- --{~:~

..,

OKcanj~

-._----t[
.
:r.

1
Leu

Lys
Phe

Asn

Trp
,

31.1.

, .

lIll().1II1.11 ".'.I"Il.'() "('.Il'I1 1I.\lI\IIIOI1l

....

I I

1 ~o-

l'

~)

I I

1.......0!
1
-

~o

:::;-'

1'0-

'14"

!
1
!

I I

ALA

Il

L-

0-

CHz

"

....

~4~===~~~~==~~.~

- ~..

-- ..

ALA

~o

~o
Ir
C

L-

L-

\. )

~====--~~--==~~~~

--

119

L-

31.2. L- () L-\.1 (I1)'), i.l\.1'l

Jd. PYR- , ALA -

. .l lIl 1 ,
.

, ,

(. )

"

'r

(.

L-

31.3,

). r

--.

(.

31.2,

).

-.

; ,

. --


(. .

29

11 --

,
.

).

1.

(.

11

31.3).

uu.

Pro/Re,

- --

(.

31.2, ).

1,

10%
1

11.

. ,

-3--5-.

( ),

>k .

11

1.

L\1_ -

320

3/

N_
H

""""'"

"

L-

H20=to I

'l

L-

It'

.......
11

. . . . . '/

..........

L----lt

lt-
-

-,

~ -

NH ..

./ 2 ,

11

/'
11

0-

------,

1 ______-11
Glu

NAO+

NAOH + +

NH+
3

/..........

2 '

.......0-

'

11

L-

I T _....u It.--- ,

~Ala

-,

/'

2 ,

11

11

2 ./ '/

11

. 31.3. L- () L- ~npaa). L-_ U


I - l 1, 2 -
11. 3 - (_ r ..l. 30).

--

(.

31.3).

-- L-r--

---L- (.

. () ()

31.12),

11 (
1)

ll.1U.W ll CKe.lea .lO

321

-,

--,
-,
(.

31.3).

L-

.-tr-I

m ,
.

30

NH'~~

i .

4 (. 31.4).

4--5-

H20~1t'

- .
4--5-

~c

888

; ' -

N-
,

r,

NS-

. ,

"~.""_

N-

~ 8888

N-.

:,
. -

,
(.

-'"" 2 ' "" '""

11

,on~ Ir............,_.-__ul

N5_~
4 8

,
).

N V88

-,

2 ,

1 I 1573

/, / 0 2

11
L-88

~I __8___8_8--1

31.4).
-

;,l.

"

(.

.1 hl

. 31.4.

L-i:l

a-.lt.
4 -
<t. . (i.1l<l .

0-

11

31.4),

- .

...

Ala~

-,

2 ,

11

/,

11

"

a-8

0-

322

31

~ peaK~
;

~.

, .

L-

. 31.5. , r
.
4 -
.

).

, ,
. ,

, .

(.

31.7;

-=-

NH;',

NS_, N1o_

.
(~

31.6)
-

..

. JO

.m..,

aamm,

-.

-,
, (.

r .

. .

L-

L-

!
~

L-

L-

~,

+-

L-

. ~, ,

, ,

X-.

, ,

60~IOOO - 1 .

,
, , 2

NS_,

. .

NI-.

n" ( -

OI ;

(.

31.5),

m60U. Ilema .uum

"

323

....... 0-

11

838

31.6.

i:lJ1
i:l.

.'1.

PLP

(.

31.7).

, ,

; , (

). ,

,
,
( ) .
.
,

-
(.

31.5).

NS,

N'o_

(.

31.6).

.
,

L- (.

-.
, ,

, t?

Jl ;
,

31.7)

Q- .

.
,

20--30
80%
"-

- ,

), -

. (.

31.8).


(. ).

L-

31.7.

i:l . -

. . .

2 . .

. . .
.

.
GIu-, --

324

31

NH+


. 2I

. . . . "

,....0-

11

- ............ .......

ft

NH+

.,..h,

7'

~)I

NADH + +

NAD+

/"

0-

11

SH

L-
-

31.8.

. .

, ,

, .

()

(3-) (.

31.9).

I88l
-8

L -

I888!

a-8

11

C/~'C/O]

[ - 2 SI

............
. . .

2(

((

HS

-8
()

11

t-

~~_ _ _--.

-v L..-..;._~"";"-'~~_-I

Iv 8l

SO) 2-

11

. . . , . . .
3

11

"8

11

. . . ,......
3

0-

11

......

. . . , . . . . 2,

"

-8

HS

. 31.9. L- ( , .,) (3 , ). J3- .


.
.

Kllma(}('-I1. .J,?l('(} : .,.,.lOm

(.

31.9)

Fe 2 +

<i=H J -S-S-~H2
HCNH,+
CH~

NAD(P)H.

325

N +

, ,

()

. .
,

ll

.'1,

bl

31.10.

()

.
-

, , .

, ;

(.

31.10).

L-

L-

. (3-)

, ,

3- ()

. ( ).

(.

3-

31.9).

L-

).

. 3-

, . . ji

; Io paHH~M

3-

. I~I

--
.

H 2S

31.9).

(.

, .
.

. .

31.2

. .

. (-).

20 - 30

160000

300

),

(.

31.2).

, .

S-.

6-

6 -.

.
(.

31.2).

Ii

(.

31.11).

31.2.

-~-
N 5, N lO--

N 5-:

29.10,

31.22
29.10,

[V

5-:

- 11

(-

2
29.8

3--

3- -

31.9

11 , lC.

L-

11

-=====~~~
02 + NH4
(. . 31.6)

'3

+ - 48

H20 J t F A D

f5-1

838

~
4

FADH 2
3 , .......0 -

838

4 8

11

NH+
1 3

" ........

,,0-

11

L-

+
Mg-ADP

NH 4

NADH + +

NAD +

-><
0

SH

j "838

4:'

(. .

31.7)

11

"~
-

"Q

8
883

. 31.11. , -.rlO4- [~10]


.

60U. .1? .wuu

327

4--L-

(.

31.12).

L-~1_-3--5-.

--L---,

:--- L-,

--.


L -lJ. 1..-S.,

JI

1
J N

,,1 _........

.
,

4-
(.

31.12).

NAD+~.H20
I 83.

, Q
,

1 L ~..-t8ItI

,
-

..........

11

-,

D++

-,

I'
-

11

/",

/0-

11

3-t-L 88

- ~ I ltt8838 I

), -.

, (
,

/",

a-K~

~ .

-,

. ,

11

. .

(.

1)

31.13):

2)

n-

, ;
;

.8

3)
4)

5)

-.
-

31.12.

-.

- -

-.

:
1- ,
. 11.

2-

328

31

10)

euJ +

\.

'%

n-PD

10)

FeJ+

......:-:....::\_..1_ _

O~

..

,_0- 2 ..... 2 ....

I~..

fi

38

......

~z...........
.. ~H2 2
...........

711

il

'0-

71

11

0~~HC~t;t!C.)r~H2,~
. J:H2""'~""o8
11
.1

-u

11

( )

11

. . . ' . . . 0..........
......

117

H3c .......c~cH2'C ......O-

11

"

CoASH

I~8'.

.
I

31.13.

........... S-

11

_ , l ~-.

. ,
(. . 31.15). - --, Glu - . L-.
1- 1. 2 - , 3- 4- 1, .

. ,

- -

. .

n-

u-m-

--

-u ,m- -

(-SH )-

. n-

. (.

31.13)

-,

n-,

. n--

(.

23).
lDl .

in vitro

,
,

1 ().

, -

m.lU.' zIl(' CKe.lcma .WU1I"'.IOlll

329


. ,
, .

31.13).

, ,

(.

31.13)

II

l-

(.

(4-5

/l00 ),

, , ,

, .

- .

. , .

6-8

n-,

1 .
6-12

. .

/100 ,

- (.

n-

, n-, N-
(. 31.14).

'!

n-

31.13).

, n-,
N-

11

. . -

). -

~.

L-

NH+
3

11

V-

0-

f'.COA.SH

Jl

HN

0-

n-

NAD+~O+
23
:

NADH+H+

NH.+ +
2 2

NADH+H+

I I

NAD+

~- L -

n-

n-

. 31.14. . n-
n-.

330

31

XVI ., 1859 .


()

~9./

-.
-

(.

31.13).

_2 -

, , ;

. 600

8VI<~ v

2-5

. 31.15.
;Ia. ;l
( ).

, ,

( lUI, )

70

, ).
, ,

. m

,

, ~

. ,


(. .

29.11).

(.
31.15) ,
(. 31.13).

.

(. . 29.12).
:
( 1,

: ),
(
11 111)
( IV V).

(.

31.3).

1 1 000 ,
1

(. .

29.12)

250/0

;
.

,

(. 31.16).
(
), (
)
,

. . 31.4

.

- .

mu.' ?? CKe,lcma ,lO

31.3.

1,

Jl

Phe-rlC

lC

331

11

Phe-rlC

lC

lC rlC

lC rlC r

IV

lC r

, 5-rlC, lC

-lC

-lC

, 5-rlC, lC

lC r

VI

VII

lC

r n-rIC

pybaT-lC

lC

VIII

1.

lC

Tyr

n-rlC
lCr

2. lC

lC

~lC Tyr
lcI r

" Jl Jl uri ., Sidbury J. .: Phenylketonuria and hyperphenylaninemia


Stanbury J. . et al. (eds): Metabolic Basis of Inherited Disease, 5th ed. McGraw-ill, 1983.


,
,

.
, ,

(.

273) in:

(. 31.16).

20

. ,
,

. ,

, ,

31.4.

. ,

(r/IOO )

(r/IOO )

1-2

15-{j3
0.3-1,8

30

300---1000
300---2000
290-550

lC

200---300

2400

332

~ CH~H-COO-

NH J

I I
I

31

Glu

-, - L-
.

2---

L-

11

- (.

31.17).

, L-

NA;c:ol

. D-

NAOH+H+
2

(.

L-
,

(.

~ 2- ~-~-t~~-

L- L--

2
2
I

--.
-

CONH 2

-.

31.18),

. 31.16.
, .

Gln -

NAD +

, ,

NADH,

, .

L- -

---

~H20

3].18)-

Gln~

Glu

,--

/{Jl

L- 4

-.
,

-,

,
. , ,

Illl Jl .

.
I
( 2 )
I

HC-N +

L-
.

31.17.

( 2 )
I

=
I

L- Q-/{. HCCKOJ1bKO : ;tjt


.

u "

11

-.....

...........

11

......

..... .....0-

11

11
.........

333
~

11
.....CHa...... ~,J:. .....o_

J ,

NADH + .
NH+

......

.....;'

I "

\.

...... ............

NAD -.......... .....

............ .....01
NH:

~ ----\:.:----......-,

~H:

0 ..........
........... ...... .....

11,2\

11

L -

NADH
NAD+

NAD+ NADH+H+

\ ct

>

L- -

NH+
1

;'

L-

..........0-

fi

.....;

--6-

-
.,

Glu
PLP

1;8: I
.

fl

-..... .....;

CoSH

./

0-

.....;' ~C

a-8

101

31.18. L- (- --, Glu-, L-).

1-
, 2 - .

-,

, ,

L- - (.

31.18)_

. r

-,

---

L-

(_

31_18).

(.

_ ,

30_13)_

Neurospora Pseudomonas,

. ,

[I4]":

r.

_
, ,

-
(_ 31.19),

.
.

12


()

. -

N-_

334

3/
NH+
1

. . . , . . 0-

Fe

~t,,<> u 1:\

1.

~N

()

N-l-

L-

NH+
1

11

\NADPH + :

. . . , . . . 0

\.

PLP)

I I

3-

3-

cr

~~,

~~,cJLNH+
11

H,~ '
I
-,/

-
, ......

..

\1

NADH+H+

NAD+

{.

~---~.

...........

. .-
.-

NADIPIH+H+

NAD(PI+

'"" ~
11

NH.

2--u. u-

2--3-

-", . . . .;'

'""'" ''"
2
~

0-

CO t + HtO

(. . 34.18)

( )

31.19. L-_ PLP -.

(.

31.19).

3-llllll

3-.

ND-

NADPH.


N-

u
~.

2 18 180

.
Ie

(. .

29).

- .

(.

31.20).

, ~ (. 31.19).

-.

, . -


J( Jl
.

mu Jl ullum

335
-5
- ,

'

- NHJ
- CO z
-

1- 1

+ CO z

- CO z

31.21. ,

-.

-.

2
. 31.20.
86'
3-
3-
(. 31.19). :l
.

-, S-
.

,

-

. ,
,

-. -
- -
. 23 JI

; ,


(, Nl). 6

L-

S- (<<

:,

NAD+

NADP+.

(.

31.22).

S-

yr

, :

1 _

S-.

8--- L-

. ,

, ( .. 3 1.23). JI

, ,

(-N-[-3-

]) .

L-

(. .
-

29).
.

,
-

cyMMape
,


-
(. 31.21).
,

(.

~1.24).

-
-,

-
(, -)
CoA-.


. . . 31.2.
1 ,

S-eJ:l, , , ,

-.

, , , N-t

, .

336
. 31.22.
.

-- CH 1 -

S-

rpynna

31

-'

+H]N-<f- H

+H]N-C-

2
I
2

..

tH

"n~

~-

~-"""L--~-----------,

It'~_'"

'~

S--L-
(" ")

N +

...... S ....... CH:1........ CH ....... tH ........ C ....... 0:1

11

V-

NH+

I
2 ,

ATP

11

L -

S--L-

~H20

~-
S-- L -

2 (\

NH +
I

-s
+

~
:1
- ............. .......
1

L-

~X;

.............
.......
:1

N +

11

N +

11

(j-838
2

NH+

t .......

:7

l
I

/0-

11

0........ .......
11

r.;

:> ....... ./

,.....0-

:1

....... , ....... 0-

-t...-

NH+

I 3
H2c~~CH ,

~
"""':1'

!'-i+i

v-

................0-

+
2

,..... .........

,.....0-

11

]
]

11
,

,.....,

/0-

11

-
-

31.23.

\1 -.

L- u-(),
<l3.1\., \.().

31.24.

mu 1l0

CKe.lema .",UIl0u.m

337

.I~.3

!0

L-, L- L-

,
.

88 Cn~1


~
(. 31.25 31.26).

t0

+ 8v

lII;'~-.~1

(),

() ().


88 . t)-8tt8~l ...

-~1

,
.

31.26,

, .

-
+ -

31.26-31.29.
. .

1)

, , .


L--

-,

. .

.
--. (

(. .

18).

- (. ),
--~- ( u) -

( ).

-),

,
,

1+


, ,

. ,


. ADP, -

-r; .

,

: 2 - (

),

( -

2)

. 31.25. u
. I-- .

).

2 -

t)r t)r-

L-

,
(, -

-). -
-
(a-).

. r
II
(

3)

llp(J

-.

-
. ,

.
.

;
,

~
;

- .

~
-,
.

,
(. 31.27)
4L: P-IIJIICJ1.

, -

138

3/

N
f

....... , ....... CH ......~ ...... 0

11

L-

v'

L-

0l'--

0t- ."

......Ct;i{ ~ ( ./

H]C/CH'l ......

0~COA.SH

~C02

~C02

11

'"

'S-

11

11

]
a--

~[2H]

0}..(2!

H2C~C ...... c,
I
S-

/C'::::::'CH/C,S_CoA
)

11

""'" .::::::./ 'S-


1
]

O--

11

11

H]C/CH'<;:H"C,S_CO

'S-

CO SH

0~[2H]
CH j

or

. . . . ./
'1

---

0~COA.SH

. ] ""'"

CH"~"f

. 31.26. . . ,
2 3 .

'1 (. .l ).

(. .

(. .

2 .

4L,

31.27)

2
- 2

2
-.

1/2
28).

,
(. 31.28)
4: -.
,
,

P-JI-. -


, L----
, 4-9

---

5L:

( 6L, :
3 1.27), ,

5: ~-
-. CoA-

(. .

6L:

28).

31.28),

~--~-JI

6V,

5V,

31.28).

-. . -

---

, .

P--.
6: -.
, -

n60.1l1 y.?lepOOIIO?O ,m QJlI11\uum

.......

339

11

~CH/

.......... S-

Mg-AOP+P,

@
Mg-ATP

11
*

...............

1
11
C~

/..,. ~CH"""'" ........... S-


- -

@!V

H20

11* ._./

/~

-"""'" . . . . 1 ~CH/''''''

11

........ S-

- -- n-

11

....... C~

~L

11

....... ......

11

S-

....... ........

31.27. (3--. L-;I (. . 31.26).


. 2 .1-2 . 20.4.

ND+-

CoA- (.

~-
( 6V, . 31.28)

7:

31.28

~-

. 2

B I2

31.28),

--

, , .

u-

,-

-.

; ,

31_29)_
B12)

7V,

8V-I0V),-B

,
~ (. 31.29)

- (

8V-I0V, . 31.28). (
1OV, _ 31.28)

--.

, -

D 20.

1 4- -

~ ,

- (. 31.29).
41: r-.

3/

51: --!J-
5V

-.

(.

31.28).

4V,

31.28).

61: a--.

2 3 -
- f3-
-
-.
, - ()
- (),

11

lc;~/c ...... 5 _
1

@rH,O

1
1 .....

11

... .......

P-~:~-

(, )
.

CoASH

11

' ....... . . . -

). 50 .
5--1
. - -

@) ~NAD+
!'-NADH+H+

11

.......... S-

~-AK
NAO+

rH'O

NAOH+H+
NH+

11

2 .......

........ ....... -

1]

....

--- -

t--

""""

("$..

a--

_/.......

11

H,C,C"""'S-co

C,......C ...... S-COA

31.28. -.
L- (. . 31.26). ---,
- -

+ ;'

11

....... S-

:I
-

.................. S-
)

@! 8 12

12H I

11

11

~S-CO

11

C ........ C ........ CH/C .......... S-COA


8
I

-''''''''

' ..... "

31.29.

-,
(. .

31.26).

L-

60ll!.,' ll lIIlOU

JI

) -

341

-.

. .

, ,

( ),

ri

, ,


( 1, . 31.26) .

1,

u- (

31.26).

. << .

,- ,

u- ..

. ,

u-,

. .

u-.

,
.

, ,

.
-- (

.
.

3, . 31.26).
- ,
.

-, ~
u-

CoA- 2 (

31.26).

2,


.
.

,
e
(, 812)
- (.

(.

31.23),

31.21)
31.26 31.29), (.

, .

, ,

-;

, ,

, (

)
. ,
.

-) (. 31.21 31.28,
,

9 V).

,2 ,

, t.:

.
. (

2 .

). -

5' -

- ,

342

31

- -,

BI2

~.

5' -

n .

B12

. .

(Wellner and Meister, 1981).

--

. .
.

B12 ,

()

BI2 (1

).

, ,

BI2

[l4C]-.

25

- I ),

5'-

100/0

Bi2

cpe~e

10000

5' .

.
.

-,

. J.

et /. Amino Acid Metabolism: ClinicaJ Chemistry


and Diagnosis, Urban and Schwarzenberg. 1981.
. J. L. Biochemistry of the sulfur-containing amino
acids, . Rev. Biochem., 1983, 52, 187.
Felig . Amino acid metabolism in , . Rev. Biochem.,
1975, 44, 933.
Friter G. W. Aminoacidurias due ( disorders of metaboJism.
(2 parts), N. Engl. J. Med., 1973, 289, 835, 895.
Paxton R., Harris R. . lsolation of rabbit liver branched chain
u-ketoacid dehydrogenase and regulation phosphorylation, J. Biol. ., 1982, 257, 14433.
Paxton R., Harris R. . Regulation of branched-chain
ketoacid dehydrogenase kinase, Arch. Biochem Biophys,
1984, 231, 48.
Rosenberg L. ., Scriver . R. Disorders of amino acid metabolism, Chapter 11. In: Metabolic Control and Disease,
dy . ., Rosenberg L. . (eds), Saunders, 1980.
Schwarz v. Clinical Companion to Biochemical Studies,
Freeman, 1978.
Stanbury J. . et /.. ( ), Metaboli.. Basis of Inherited Disease, 5th ed.~ McGraw-ill, 1983.
Wellner D., Meister . survey ofinbom errors ofamino acid
metabolism and transport in , . Rev. Biochem.,
1981, 50, 911.

32


Ie I

,
.

.

,

- r

, ,
, , ~,

(.
. 33).

, ,

Q ,

. ,

--

, ;

- (.

32.1)

- -

, ---. -

. Ha~OHe,

. --


- ;
- (

, ,
.

J3-

),
.

ffi


, .

,
' ,

33.

4, 5

(. .

35).

( -

);

5-

(.

), ,

(-).

(.

32.2).

. ,

lC

. ,

344

32

--...j.~

li-

I
(fH 2) 2

00-

/.

(
) 2 ~'''C
- .. 4
J 2
~

'~

32.1.

-8 _

11

,-

(N-

S-.

Mg-ATP-t- CoASH
Mg-ADP+P'1

11

OC"s-

CoASH

-t
~

32.2.

tococcus faecalis

67%

39-50% StrepStaphylococcus aureus.

11
, /C~, ,..0-

11

, D-;

-I
P-l ;

(. ) (.
.

17.6).

llll l'-'IU1I',lOm l'm'lIUUJuIII

345

-
- ;

(. .
(. 32.3).

35.17),

S-Jf)

, N-
:

S- + -+
S- + .

P-.iI.

-,

COZ'

--


,
, , ,

-.

JJ-E I

N~H;

-
(. 32.3).
(N-
--l-- L-, .

32.3)

~H

0""""'"
'""
2
11

'

,
(

, ). ,
,
.

---

. ,

'
,
.

in vitro.

. ,
,

(. .

7).

- L-
,
:

. 32.3. , .
.1 ,
.

+ L- + - -+ +
PP + .
j

. ).)

346

32

.;.

, .

yr . G
-
XapTI.lY

- (. .

31).

. - ~
( )

(. .

D-

35).

0-

L-

L-

'
(-).

u.

31.

S-

, im
.
,

s-

(y--L-, .

32.3) -

. .

1,3-

, (.
.

32.6).

; .

L-'

, ;


, -, .

l? (. .

). L-


L-.
( )
(. . 29.10)

aPOMa~x

L-.
,
5-

'

, .

, , (.
). in vitro in vivo


-.
(. .

25).

llu Q_'l.Il0lI nUQJlull1l11

347

I
r,

"

-,),-

novan..-

32.4.

. . '.10 .

u. ll JI Ull1 11 .
. " ;~ .1.

), (.

32.3).

l-,

, , .

. rI

3-

50 /100 ,


, .

, ., ,

32.11)

Strepto-

(.

(.

myces.

32.4),

) .

30)

(. .

( . ) ~

(.

32.6).

( )

(, -

(.

32.5).

0,5

.

.

.
,

.

348
+

HfJ.......

.......C~

;.

32

....... N

rr--------~A~------~'r~--------~A~--------~,

2.

1,- ()

... H 1N,

....... 1 . . . . .

2.

.......~,
CH 1

. . . . . CHz.........

/CHz.......

2..

';

"CH~

1,4-
()

'......
l.

CH Z

.............
:t

~'NH;

__------~~------~'r----------------------\Ir--------------~

(6)

' ......
l.

1,5-

()

_ 32.5. r .1. . ~J1


() (). r i.t (.).

32.6

(),

m .
,

L-

(f , . .

30),

--

L-

ss-

(
).
-

. - , ,


, . .



~-

(
,

S-

s-

10 ) ,

'

).
S--

s-

- (.

10-200 .

).

(1-2

32.6)

s-

S-

llll(' UAlUlIOKUCJlOm n1lll

32.7

.
,
,

~ ~.

- .

NH ~

2 ,

. -

349

5-

Mg-ATP

+N
I

+N~~

Jo-I

L-

t r::

S-

S-

N
~N+

1j I

+N~~

S-

~I
s
+

+H~
N~N~Nj

S-

32.6. . -
, 8 .

l50

32

:;>

+N~N~N~N+
.

), (

).
-

2.

~02

2.02.

, ,

~N+

/J

HsN~N~NH;

1%

~.02

, .

Z0 2

60%.

/J

+H"N~NH+
3

55-

N-


(.

NH; + 0 2
.

32.7.

~JlJ lJa.

/\

.1

i:lJl~

l-"\1

32.8,

4).

N32.8, 3)

5-

. .

Dl

29.11).

N-

(. 32.8,
5- (.
(.

(.

5)
32.8,

6).

5-

.
5- 5 () (. 32.8,

, ,

1) -

5- -90,

,
(, ), .

5-

L-

(6 /);

(70%)

5-

, (.
32.8, 2).
. ,
,

(.

32.8).

,
,

5-

, -3-,

- .

.
,

-3-.

2-8

5-r:

u1l' aMUllOtlC.llJlll fJ nliJlllllll "pOOJ'"mbl

351

NH'"
I
:1

2-

5-

0~C01

..

:,>, '~3
2

~0

o. 5- (~)
-.

I I

2.' ..... 0-

~
/

-.
INH.+)

.... ."
~

COSH

5-'

I -

NH;

5-

::1:~~~: ~-:~"~,&,.,
-. ~NH)

INH.+)

N-

..... :,> . . . . / -

5--3-

5--3-

. . . .:.>"...-:~,r.. . . ]
I I
:,>

RR

RR

"

(N--5--

"

32.8.

[NH '4] -

u~ - Oll~IfJ1]. T~KCTC

ll.

( ,

(, ).

It

, : .

5,6-

;
2 2 ,

~ ~ (

, )_

352

32

JC['C

Oo-

I ~

NHt

I3!<D
O~y'~Y;a
o~ ;;~

(3,4-)

Ill<D

~oo-

'\

...........

O~:C~

./'
I :~o-

OH

~
5-S~ :1~~I
:

~o-

0-

NH:t

: 1

+N

/ + H1 N

sJ

_~~~

O~

""

5-S--

'

O~

I I,~ ~ ~ ~ ~ ~ ~ , 1@

~: -s~o

/"

0-

t~~i

O~
~l

/-5~

- , -

on

............

0-

'"

I I

. 32.9. .
. "l .
. . 1. , .1 .

32.9


.
-

, (. ).


8

, ,
OJ m mrn

353

MOyr : .

, , -,

, .

(.

32.10).

, .

I- -,
, , , -

, ,
.

,
- .

r , ll I 11IpO
, .

in vitro ,

.
, ,

I
~

NH+
1 3

.... .... .... ,

11

l-

- ~ .-
r88

, -

,
.

I
~

in vitro

NH+
I

CH ....C!i

--------....,~L
888

. ,

reTepQ-

r ( -

HO~
2

' .....NH+
3

)
. ,

- ( ),

-. ,
,
.


- . (

)
,

N- -

";:'"


,
.

. 12 1573

s-

'--------....

S-

U/, ...................

. 32.10.

.
PLPu.

354

32

. r_ 8IIII8

-N-

s-

,
(.

32.1 ).

lC

(. .

46).


, ;


,
.

-
L-;

-
- (.

32.12).


, .
L-

y-Jt,
,

-m
(.

32.5). J:

,
.
. , ,
M


(. 32.11).

N .


. ,

(. 32.6)
-
,
.

- (.

-IIIIII, pr ..

(-r,

32.12)

Ji) (IC

--;

). (

-.

).

L-,

()
- -

/ NH2

--7';::'=====~"~~" H1N +=,

HNCHz- -

8
()

() ( "
")

S --

'IA~~--~--~

S-

~O

N-C
HN=<

r--------...,

_.....:====:;;:===:......_

N- z
I

NH

HN =

~CH1-COO

32.11.

llu .III1\U.lOm nu.1UllIllIl'

355

I
-~- N +
2
I
2

I
-

L-
2

,
I
2
I

+ N- 2 -,- 2 -

,-

(01

I
2

,-

1
I
-

NAD+

NADH+H+

7,

NAD+
- 8
.

32.12.

..

NADH+H+

-. - -- -. -- -.

yum ( )
2 2.

PLP -

, ,

,. : 1(
- -.

JI
-,
,

.

l(. l(
, ,

Stanbury J. . et /. (), he Metabolic Basis of Inherited Disease, 5th ed., McGraw-ill, 1983.
. W., . Polyamines. Annu. Rev. Biochem.,
1984, 53, 749.


.
.
,
.

. -,
.
,

(.
.

6).
.

(
, ,

. .).

- ,
.

,
;

,
.

. -


.
u , -

11

d,
(.

33.1).

11

. . .

=
6

eHC-

u.

II'v '

7-

H~

IC 20 H,.N.)

-=

11

11
~N,
,
HC-C~
-
- 11II1f3

, ,
-

, -

HC-C~ ...... =
11
N
1]

, .


, ;
,

6.2.

. 33.1. .
1, . 111 IV.
1, 2, 3, 4, 5, 6, 7, 8. -

13.

357

llthl U .llt IlU?\fn

IV

11

PIIC.

1934 .

33.2.

(. 33.2).
. 33.2, 33.3 33.4;
: () = - 2 ; (
)

= -

CH~CH2COOH; ()

= - CH J ; V

) = - = 2

(

IV

111.

).

.
. - ,

13 000,

1 -

.
. - ;

.
,

u -

111.

1.
1 111, 111
(. 33.3).
, ;. 33.4,
111 (

111).
IX,
,

. .
.

" -


, . 33.1.

"

: (

-,

)
,

=r-

111
.

33.3.

111

358

Fe1+

I I

..

Fe1'

111 (l)
(, )

33.4.

33

( )

33.6).
(<< - )

(, ~, , ),

--~- ,

-
() (. 33.5).
-. ,

.

(,

,
1 111;
,
111, ,
( )

),

.
.
-


(. 33.5).

111.

- Z-

.
(. . )

io

.
111, ,
. -

(.

~Hz

(""

CH z

CoASH

:~-':'-~~;

..

:' -

,+
:

.. ~ _______ J

H-C-NH 2

CH z

CH z

1,_-----'

6-

--13-

/..

2
I

NH z

I
2

CH z

1--------

CH z
=

C:H z

_,
-

33.5.

CH z
I

--
11

11

./'-/
2
N

'

NH 2

NH

6 -
.

_~========:::;-_~..

CH z

=O----H~C-H
'_.

-~~---. h-t-Nz
I

- 9-NH1

CH z

0 1

I
=
I

(
)

. - . -
.

uu

If",m

359

-I-,

-,

in vitro

1 (.

or-IU-

33.6).

111,

----------.
,--_ _ _ _ _ _-.r8 r. 1

r ) {

r,

111-

--

1'=/
I/~,
"

.UL

..... "

11

--

I'~/

I,,~,

--

HJ

JJ

) .

11

~ ~ .

--

'~/I

/~. . . . I

111

111

(), .

().

-,
1
1 (. 33.7).

CHJ

Dl' 11 ..... ..... 11 m 11


-- HJC--C
1
1
1
1
11

11

CHJ

/~,..!
m

""'......

11

11

HJ

I
--

'="1

1.
,
, .. .

C~

CH J

HJ --
C11 ...... C.........

11 .. I
(;
.

( )

1) (. 33.6).


111,

111

111 ,

III,
III. , , .
.

33.6.

IV

11

.,

IV

11

111
~

V,

'\

111
-

Vett

33.7.

..

11

1\/

111

111
IV

11

111

111

111

lClC ( ). -
. -

. -

360

,

, ,

111. , -,
1
.

II

(),

. ,

- ,
(. 33.5);

(-). ,

- - (.
33.4). ,

, .

- .

.,


. 33.8.

-.

. 33.9. ,

1118

V 8 1

1-

88

.n
::

111

111

tS

::::r

111

~.

42

~I

..-
.......
----------,-

::I:

s
s

88

no-4

111

Q..
:[

no

r"K-------------'-

no

111

}:'U

in vitro

tS

33.8.

UUII 1I ;ll nu:um

361

~ .

-.
,

1 ),

-450.

- .

-450,

-
-

n~

-
.
-;
_______

,, __

"

"

Fe2+~

in vivo


(
).
- ,
-

111

-;

I
I

111

, .


111



. ,


, .

111

3,0--4,5 ;

,
.


, ,


5/0- (.

;I] t-e------j

. 33.9. lt . Jl
-.
- <1
.
() .

33.10).

400

,
.
.
.

550

592

, -

362

..
;

u 33.1.

l '

10
):

3
j

i'-

,/

/'

'"

'

(.

400

an

-1)

(/

uw
(/l

3R)

33

f1

I -

500

600

4000
1500
50
300

"

l'

5
50

3
80

120

( ), RnOl, 3

Meyer u. ., Schmid R. he porphyrias. In: The Metabolic asis


Inherited Disease, 4th ed. Stanbury J. ., Wyngaarden J. ., Fredrickson
D. S. (eds). McGraw-iII, 1978.

700

n""

(0.01 % 5%- HCl).

33.10.

. 33.1

.

67 -I;
1 14 .-I,

II. ,


.
;

.,

1 111

(-

=) (
) ( - 2 - )
.

.

, .

645, 591

540 ),

540

580

; np

.


. :
,

np

-I.

111-53

1 111

,

. , ,

rpynny
, M
l.
llllblll,
- ..

, .


.

.

(.

33.2).

nu.m

33.2.

II

l'

(
)

363

ecJ:

-I-

()

- 111-

-I-

II

-
(?)-

(?)

l' , , Meyes U. ., Schrnid R. porphyrias. In: The Metabolic 8asis Inherited Disease, 4th ed.
Stanbury J. ., Wyngaarden J. ., Fredrickson D. S. (eds). Gw-ill, 1978.
,

- -

-.

.
, .

-I- 1

, , ,

-~ ,

.
. 33.11.
(000)

x - 5-

moCtI mel

_-I-.
,

- .

-I-

500/0

llJl .
QeIIW,

.

....

.' IIIIII


,
.
,

lI <:,

, ,
,

~
().
,
, ,
, i
IC;
-,
~ Ii
, JIlO R~ . ,
,

JIl(, ,

-450,
. ,

, n
; - 500/0.
-

364

33

Fe2~ I I

t
t

RR

..

I)

t RR
}

________________________

AIik-<... 1

. 33.11. , , ,
, 1(0 . -- . (
ufm

L., Merver

S. Biochemical defects

two types of

hum

hepatic prphyria. N.

gl.

J. Med.

1970:283:954.)


~I ~ -,

1--

,
111,

-
.

aR

100:1.

1 111

1,

- .

( ).

, ,

-III-

-I

111 -

III,

1.

. ,

, ,

; -

, -

Jlill U .,III(! lJ?IIl'1tnl

365

, .

(. ).
, ,

- ,

--

.

,

111 IX.
111

-.

. ,

-,

.

( )
-.

, ,

; ~

, -

-
,
.
,
, -

<:> ;
.

111;

, -,

~ .

. ,


IX, (

. ,

) (

) .
, ,
IX,

-.
,

()

,
, ,

. ,

, .

, ,
, -

, -,
- .

366

, -
,

1-2'108

. ,

70 6
.
()

;
.

33

- -.


- ( );

. - c~ceMa

.
.

33.12,
NADPH;

NADPH

11.

--+

V-NADPH
f'...NADP

NADPH
(

NADP

F + )
} (BA)(aeM~

...

'-

33.12.

-lC . ( ~

McDonough

. .

Porphyrins.

l .

(ed.). Academtc Press, 1978.)

Schmid R.,

num

-.

-,

367

llJllle IX-.

IX-a

JI

, . ,

n
IX-a (. 33.12).

(. .

111

IV

1 35
.


250-350 .


in vivo:

) ,
. ,
,

.

mo (. 33.13).

, , .

2)

3)

UD-I"IO

I .

1)

33.14.

,
.

UD-,
(. 33.14)

,
- . 100

(.

25

. &Ol"

33.14).

UD-

; ,

, .

eH~p .

, , -

368

33

- OOCICH 2 0I.C - 0-

"

11

--

CICH 2 0I.COO -

= = -id- -(:j-c =tzJ-


.

( ).
.

33.13.

UD-,

UD-

----(---,__\,.....----I..

~ UD-8

2NADH + 2+
UDP

UDP-n

UDP

UD-

UD-Q - - - - - - - - -.......

..

+
UDP

2 - - - - - - - - - - - ' . . . .
+

33.14.

. UD-
. UD-.

> .
(


97%)

(IJ-mo);

(.

33.15).

"

. ,

. .

nu.em

H2C~OH

~ HN~OH

M
QNH
-

HC

N
H2C-QCH2

NH

_'

~O~~

HN

H2C~CH2

( L -v)

(440N 4)
.

33.15.

H2Cg~

M~N ~ t~
p~

'~

369

( L -)

, ,
.

Rx ll

( )

,
, << II.

(.

33.15).

.
,

,

. -
;

.

.
JIJ{PIU~

, ~
1 {1 00 Ji (17,1 - I ),

, Q

.

, ,

8, Il
- (nlpoaI) 611-

JIII,

.
,

, n

. ,
, l
.

. ~ .

- ':'

; , <<oI .

JIII - n
().

-
u,-

),

.. (h

- .
-

) . ()

370

33

lL1Illp-UD-l'JIIO
.

, IGO

( ),

15

. ,

.-

- 1

.
20 blr/lOO .
. - aJI 11.
, -,


.
m 20 blr/lOO
,
. ,
ll

/l00 ,

68,4

/)

. , ,


~ ,

; ,

UDP-l'IOR, 8Jlll.,

IOJJ801 yw i:

,

.
, -,
,
.

UDP-l'JIIO
, ,

,

UD- . -

, 01

(.


.
. ..
,

(20-25 /l00 ).

.( ). ,

).

11

, ck,

. ,

I' ,

( );

, -, ,

8JdI lllUl llllp8

, .
. -ihlpa 1;


-uD-l'JO....
, ,

), , -, -

1-

num

371

(~
,~

",

0-4

RnII

~~~:::J--"'"

tc

08~,

~'W8 ~lAen
;
oTcyrCTByeT

, ()

f'

. 33.16. - . ,
- . (, , . . et al. Physisians

Handbook, 21st ed. Lange, 1985.)


. TOK~Ka. nmeJIRIUI.
aJI
,
(,

m ;

.

. ll ll (

~, ~ ,
),
, Anianita.

-).

ll'

8 .

.
, ,

1dOl JI

,
.

, -

372

33

(, )

. ,
Ji -

. ,


.
, .

33.16,

r .
, , ,

' ,

",

8JI

,
,

0,64 ; 4

I ).
' ,

,
.

)
.

Ballersby . R. el /. Biosynthesis of the pigments of life: Formation of the macrocycle, Nature, 1980, 285, 17.
Berk . D. el /. Disorders ofbilirubin metabolism. In: Metabolic Control and Disease, 8th ed., Bondy . ., Rosenberg
L. . (eds), Saunders, 1980.
Kappas ., Sassa S., Anderson . . porphyrias. In: The
. Metabolic Basis of Inherited Disease, 5th ed., Stanbury,
J. . et al. (eds), McGraw-iII, 1983.
mrg R., Legge J. W. Hematin Compounds and il Pigments, Interscience, 1949.
Schmid R., McDonough . . Formation and metabolism ofbile
pigments in vivo. In: The Porphyrins, Dolphin . (ed.),
Academic Press, 1978.
Tschudy D. ., Lamon J. . Porphyrin metabolism and the
porphyrias. In: Metabolic Control and Disease, 8th ed.,
Bondy . ., Rosenberg L. . (eds), Saunders, 1980.
Walson . J. Gold from dross: The first century of the urobilinoids, Ann. Intern. Med., 1969, 70, 839.

-:

Ara
Arg
Asn
Asp

----

Cer
(Q)

(Q)

,4-

dA
dC
dG
dT
dTMP
dUMP
(Enz)

2,-

(-

")"

~-5' -
-5' -
f
-

FAD

FADH2

FMN
Fuc
G
AGO

Ala
ALA

GalCer
GalNAC
GDP
GlcCer
GlcNAC
Gln
Glu
Gly

. (, . .) (, . .)

(-

(-

N-

N-

374

NADPH

NeuAc
P

HbF
HbS

His

4-

IDP

IMP
ITP
IUB

Kd
Kcq

Leu

Lys

Man
Met
NAD+
NADH

Phe
PPj
Pro
PRPP
QIO

Ser
Thr
Trp
TTFA

Tyr

5--l-

UDP
UDPGal
UDPGlc
UMP
UTP

AJ.1

()

NADP+

(i)

()

1.

R .


, ,

2.

10
10

12

12
12
12

3.

4.

9
9
9
9
9
1

5
7

1.

11
11

13
13
16
19
19
20
21

_.

21

21
21
21
22

33
33
33
33
34
35
35
36
37

24
24
24
28
29

32

38
40
41

376

O.>.IaB.I("/I('

5.

: .

42
42
42
42
43
45
47
48
48


49

50

51

6.

: .

7.

: .






()


8.

: .

52
52
52
52
53
55
62
63
63
63
63
65
66
67
68
69
71
71
72
73
73
75
76
76
76
76


.


-
, -

9.

: u.

77
77
77
78
79
81
81
82
83
83
84
84
85
87
88
90
90
91

91

91

91

93

94
94

ll

10.

95
97

: .

98
98
98
98

11.

11.

377

99
102
104
108
110
III
III
III
III
III
112

12.

orlW .

113
116
117
118
118
118

- ,
-
118
, -

119
126

13.

. -

14.

15.




()

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