Uroporphyrinogen-III-Synthase

The uroporphyrinogen III synthase ( URO3S ) is the enzyme in animals that catalyzes the cyclization of Hydroxymethylbilan to uroporphyrinogen III, a partial step in the Porphyrinbiosynthese. In human URO3S can be found in all tissue types. Mutations in the gene can UROS and this lead to URO3S deficiency to Günther's disease.

Catalyzed reaction

⇒

Hydroxymethylbilan cyclized to uroporphyrinogen III.

Human Genome Organisation Lyase
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