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6 Cards in this Set

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accumulation of heme synthesis intermediates results in
porphyrias
rate-limiting enzyme in heme synthesis
δ-aminolevolinic acid synthetase [ALA synthetase] (succinyl-CoA + glycine --> δ-aminolevolinic acid)
lead poisoning and heme synthesis:

enzymes affected/accumulated substrates
ALA dehydrase/ALA

ferrocheltase/coproporhyrin
acute intermittent porphyria:

deficient enzyme
accumulated substrates (2)
porphobilinogen deaminase (aka hydroxymethylbilane synthase, uroporphyrinogen I synthase)

porphobilinogen
ALA
porphyria cutanea tarda:

deficient enzyme
accumulated substrate
uroporphyrinogen decarboxylase

uroporphyrin (gives urine a tea color)
signs of porphyrias
Painful abdomen
Pink urine
Polyneuropathy
Psychological disturbances
Precipitated by drugs (e.g., barbiturates, alcohol)