Uroporphyrinogen III Synthetase
Synonyms
Cosynthase
Cosynthetase, Uroporphyrinogen III
Hydroxymethylbilane hydro-lyase (cyclizing)
Isomerase, Uroporphyrinogen
Synthase, Uroporphyrinogen-III
Synthetase, Uroporphyrinogen III
Uroporphyrinogen III Cosynthetase
Uroporphyrinogen III Synthase
Uroporphyrinogen Isomerase
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.
See Also