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PORPHYRINS AND BILE PIGMENTS

PORPHYRINS ARE CYCLIC COMPOUNDS FORMED BY THE


LINKAGE OF 4 PYRROLE RINGS THROUGH METHENYL
BRIDGES ( - HC = )

A CHARACTERISTIC PROPERTY OF THE PORPHYRINS IS


THE FORMATION OF COMPLEXES WITH METAL IONS
BOUND TO THE NITROGEN ATOM OF THE PYRROLE RINGS,
SUCH AS :
- IRON PORPHYRIN : HEME ( HEMOPROTEIN : HEMOGLO-
BIN, MYOGLOBIN, CYTOCHROME, CATALASE, PER-
OXIDASE, TRYPTOPHAN PYRROLASE).
- MAGNESIUM PORPHYRIN : CHLOROPHYLL.
BIOSYNTHESIS OF PORPHYRINS :
- SUCCINYL-CoA + GLYCINE  ALA / AMLEV.
ENZYME : ALA SYNTHASE, RATE-CONTROLLING ENZYME,
COENZYME : PYRIDOXAL PHOSPHATE.
- 2 ALA  2 H2O + PORPHOBILINOGEN (PBG)
ENZYME : ALA DEHYDRATASE:
Zn CONTAINING ENZYME,
SENSITIVE TO INHIBITION BY LEAD.
- 4 PBG  UROPORPHYRINOGEN  COPROPORPHYRINOGEN
 PROTOPORPHYRINOGEN  PROTOPORPHYRIN 
FERROPROTOPORPHYRIN ( HEME).
-TERMINAL ENZYME: FERROCHELATASE/HEME SYNTHASE.
THE CHARACTERISTIC OF PORPHYRIN :
- COLORED SUBSTANCE,
- RED FLUORESCENCE,
- SORET BAND (ABSORPTION CURVE IN THE UV REGION
400 nm.).

PORPHYRIA : A GROUP OF DISORDERS DUE TO ABNORMAL-


ITIES IN THE PATHWAY OF BIOSYNTHESIS OF HEME , CAN
BE GENETIC OR ACQUIRED WITH SYMPTOMS :
- PHOTOSENSITIVITY,
- NEUROPSYCHIATRIC.
CATABOLISM OF HEME.
- UNDER PHYSIOLOGIC CONDITIONS IN THE HUMAN
ADULT, 1 – 2 x 10 8 ERYTHROCYTE ARE DESTROYED
PER HOUR – APPROXIMATELY 6 GRAM OF Hb / DAY.

- Hb  HEME + GLOBIN.
- HEME  HEMIN  HEME  BILIVERDIN  BILIRUBIN.
- BILIRUBIN ( RES )  BLOOD (BILIRUBIN-ALBUMIN)
 LIVER. (BILIRUBIN  BILIRUBIN DIGLUCURONIDE
 BILE( BILE PIGMENTS)  GI TRACT : TERMINAL
ILEUM  LARGE INTESTINE : THE GLUCURONIDES
ARE REMOVED BY SPECIFIC BACTERIAL ENZYMES
(BETA GLUCURONIDASES), AND BILIRUBIN / BILE
PIGMENT IS REDUCED BY THE FECAL FLORA TO A
GROUP OF COLORLESS TETRAPYRROLIC COMPOUNDS
(UROBILINOGENS). IN THE TERMINAL ILEUM AND
LARGE INTESTINE, A SMALL FRACTION OF UROBILI-
NOGEN IS REABSORBED AND REEXCRETED THROUGH
THE LIVER. (ENTEROHEPATIC UROBILINOGEN CYCLE).
- MOST OF THE COLORLESS UROBILINOGENS FORMED
IN THE COLON ARE OXIDIZED TO UROBILINS ( CO-
LORED COMPOUNDS) AND ARE EXCRETED IN THE
FECES.

JAUNDICE / ICTERUS IS A SYMPTOM CAUSED BY


INCREASE CONCENTRATION OF BILIRUBIN IN THE
BLOOD.