Enzyme deficiency in Acute intermittent porphyria is
B. i.e. (Uroporphyrinogen- I synthase) (286 - Harper 27th)Enzyme involvedPorphyria* ALA synthase* Uroporphyrinogen I synthase- X- linked sideroblastic anemia- Acute Intermittent porphyria* Uroporphyrinogen III synthase- Congenital erythropoietic porphyria* Uroporphyrinogen decarboxylase- Porphyria cutanea tarda* ALA synthase is the rate controlling step in hepatic porphyrin biosynthesis*** Porphyrins are synthesized mainly in Liver and bone marrow*** The conversion of uroporphyrinogen III to coproporphyrinogen III is an example of - Decarboxylation*** Prophobilinogen in urine produces pink colour with Ehrlich's aldehyde reagent**
ALA synthase
Uroporphyrinogen I- synthase
Uroporphyrinogen III synthase
Uroporphyrinogen decarboxylase
Miscellaneous (Bio-Chemistry)