Definitions for uroporphyrinogen iii synthetase

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U.S. National Library of Medicine

  1. Uroporphyrinogen III Synthetase

    An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.

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