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