Uroporphyrinogen III Synthetase |
|
| Cosynthetase, Uroporphyrinogen III; Isomerase, Uroporphyrinogen; Synthase, Uroporphyrinogen-III; Synthetase, Uroporphyrinogen III; 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. | |