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6 Cards in this Set
- Front
- Back
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accumulation of heme synthesis intermediates results in
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porphyrias
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rate-limiting enzyme in heme synthesis
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δ-aminolevolinic acid synthetase [ALA synthetase] (succinyl-CoA + glycine --> δ-aminolevolinic acid)
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lead poisoning and heme synthesis:
enzymes affected/accumulated substrates |
ALA dehydrase/ALA
ferrocheltase/coproporhyrin |
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acute intermittent porphyria:
deficient enzyme accumulated substrates (2) |
porphobilinogen deaminase (aka hydroxymethylbilane synthase, uroporphyrinogen I synthase)
porphobilinogen ALA |
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porphyria cutanea tarda:
deficient enzyme accumulated substrate |
uroporphyrinogen decarboxylase
uroporphyrin (gives urine a tea color) |
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signs of porphyrias
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Painful abdomen
Pink urine Polyneuropathy Psychological disturbances Precipitated by drugs (e.g., barbiturates, alcohol) |
