• C H A P T E R • 18 •

UREA CYCLE
•

• • • • • • • • • • • •

UREA CYCLE
Function: To provide a route to dispose of the amino groups
from amino acids during their metabolism.
Location: Liver, kidney
Connections: From amino groups of amino acids through gluta-
mate and glutamate dehydrogenase
From amino groups of amino acids through aspartate and ar-
gininosuccinate synthase
From ammonia through carbamoyl phosphate synthetase
To urea
Regulation: Primarily by availability of amino groups and ammonia
Equation:

NH4  CO2  Asp  2ATP ¡

NH2C(“O)NH2  fumarate  2ADP  2Pi

Glu  NAD  CO2  Asp  2ATP ¡

NH2C(“O)NH2  -ketoglutarate  fumarate  2ADP  2Pi

(See Fig. 18-1.)

225

BG McGraw-Hill: Gilbert, Basic Concepts in Biochemistry, JN 5036

• 226 • Basic Concepts in Biochemistry

ketoacid

amino acid
Glutamate 
amino groups into NAD
glutamate
glutamate via
dehydrogenase NADH
transamination
2-ketoglutarate 
NH4  CO2  2ATP

carbamoyl phosphate
synthetase
O O

—
—


H2N— C — NH2 NH3 H2N— C — O — P
urea carbamoyl phosphate
—
—
—
—
—
NH2 omithine NH2
—

—
C — NH2 citrulline C—O
 —
—

arginine
NH NH
—
—

—
— —
—
—
— —
— 
argininosuccinate  CO2
H3N —
NH2
 ATP CO
—

 CO2 2
O2 C
C — NH — aspartate
ketoacid

CO
—

amino groups into

CO2 2
aspartate via
NH
fumarate transamination
—

—
— amino acid
Oxaloacetate
—
—

Figure 18-1 The Urea Cycle

BG McGraw-Hill: Gilbert, Basic Concepts in Biochemistry, JN 5036