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What is Enzyme deficiency in Acute intermittent porphyria is and explain in detail?
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**