The protein encoded by this gene catalyzes the fourth step of porphyrin biosynthesis in the heme biosynthetic pathway. Defects in this gene cause congenital erythropoietic porphyria (Gunther's disease).
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Protein Aliases: hydroxymethylbilane hydrolyase; Hydroxymethylbilane hydrolyase [cyclizing]; urogen III synthase; UROIIIS; uroporphyrinogen III co-synthase; Uroporphyrinogen-III cosynthase; Uroporphyrinogen-III synthase
Gene Aliases: AI415298; UROIIIS; UROS; Uros3
Molecular Function: hydrolase