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17 Cards in this Set
- Front
- Back
Question
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Answer
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Lead poisoning blocks heme synthesis at what 3 sites?
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1. ALA synthetase 2. ALA dehydrase 3. ferrochelatase
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What is the committed step in heme synthesis?
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ALA synthase
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What enzyme converts Succinyl CoA + Glycine --> delta-aminolevolinic acid (ALA)?
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ALA synthase
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What enzyme converts delta-aminolevolinic acid --> prophobilinogen?
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ALA dehydrase
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What enzyme converts Porphobilinogen --> pre-uroporphyrinogen?
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uroporphyrinogen I synthetase
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What is the disease called when there is a buildup of porphobilinogen & delta-ALA in the urine?
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acute intermittent porphyria
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What enzyme converts Uroporphyrinogen III --> Coproporphyrinogen?
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uroporphyrinogen decarboxylase
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What is the disease called when there is a buildup of uroporphyrinogen III?
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porphyria cutanea tarda
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What enzyme converts Protoporphyrin --> heme?
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ferrochelatase
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What co-factor is needed to convert Protoporphyrin --> heme?
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Fe2+
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What builds up in the urine in Lead poisoning?
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1. Coproporphyrin 2. ALA
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What enzyme is deficient in Acute Intermittent porphyria?
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uroporphyrinogen I synthetase
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What enzyme is deficient in Porphyria cutanea tarda?
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uroporphyrinogen decarboxylase
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What builds up in Porphyria cutanea tarda?
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uroporphyrin in the urine (tea-colored)
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What is the symptom of Porphyria cutanea tarda?
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photosensitivity
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What are the five GENERAL symptoms of Porphyrias?
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1. Painful abdomen 2. Pink urine 3. Polyneuropathy 4. Psychological disturbances 5. Precipitated by drugs
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