<prompt> Enzyme deficiency in Acute intermittent porphyria isALA synthaseUroporphyrinogen I- synthaseUroporphyrinogen III synthaseUroporphyrinogen decarboxylase  <response>: B. i.e. Uroporphyrinogen- I synthase 286 - Harper 27thEnzyme 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 Ehrlichs aldehyde reagent 