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Glossary of FA Biochemistry Heme Synthesis

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

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