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.
Also Known As:
Uroporphyrinogen-III Synthase; Synthetase, Uroporphyrinogen III; Cosynthetase, Uroporphyrinogen III; Isomerase, Uroporphyrinogen; Synthase, Uroporphyrinogen-III; Uroporphyrinogen III Synthase; Cosynthase; Uroporphyrinogen III Cosynthetase; Uroporphyrinogen Isomerase; Hydroxymethylbilane hydro-lyase (cyclizing)