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