uroporphyrinogen I synthetase
- uroporphyrinogen I synthetase
-
An enzyme of
haem biosynthesis that is defective in the inherited (autosomal dominant) disease,
acute intermittent porphyria. UP I is isomerised to UP III by UP III synthetase, defective in the
autosomal recessive disease, congenital erythropoietic porphyria.
Dictionary of molecular biology.
2004.
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