Uroporphyrinogen III Synthetase
Cosynthetase, Uroporphyrinogen III
Hydroxymethylbilane hydro-lyase (cyclizing)
Synthetase, Uroporphyrinogen III
Uroporphyrinogen III Cosynthetase
Uroporphyrinogen III Synthase
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.