Hydroxymethylbilane Synthase

Synonyms

Ammonia-Lyase, Porphobilinogen

Deaminase, Porphobilinogen

Hydroxymethylbilane Synthetase

Porphobilinogen Ammonia Lyase

Porphobilinogen Ammonia-Lyase

Porphobilinogen Deaminase

Porphobilinogen ammonia-lyase (polymerizing)

Pre uroporphyrinogen Synthetase

Pre-uroporphyrinogen Synthetase

Preuroporphyrinogen Synthetase

Synthase, Hydroxymethylbilane

Synthase, Uroporphyrinogen I

Synthetase, Hydroxymethylbilane

Synthetase, Pre-uroporphyrinogen

Synthetase, Preuroporphyrinogen

Uroporphyrinogen I Synthase

An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8

See Also