Heme synthesis (First Aid) – Flashcards
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initial reactants
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glycine, succinyl-CoA
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first enzyme product location cofactor inhibited by
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enzyme: delta-aminolevulinic acid (delta ALA) synthase product: delta ALA location: mitochondria cofactor: B6 inhibited by: glucose, heme
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sideroblastic anemia
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delta-ALA synthase deficiency
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rate-limiting step
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delta-ALA synthase
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lead poisoning
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delta-ALA dehydratase deficiency (2nd step: delta ALA -> porphobilinogen) or ferrochelatase deficiency (last step: protoporphyrin + Fe2+ -> heme)
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acute intermittent porphyria
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porphobilinogen deaminase deficiency (porphobilinogen -> hydroxymethylbilane)
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porphyria cutanea tarda
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uroporphyrinogen decarboxylase deficiency (uroporphyrinogen III -> coproporphyrinogen III)
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enzymes that act in mitochondria
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delta-ALA synthase ferrochelatase (first and last)
