Hydroxymethylbilane Synthase |
| Hydroxymethylbilane Synthetase; Pre-uroporphyrinogen Synthetase; Preuroporphyrinogen Synthetase; Ammonia-Lyase, Porphobilinogen; Deaminase, Porphobilinogen; Porphobilinogen Ammonia Lyase; Pre uroporphyrinogen Synthetase; Synthase, Hydroxymethylbilane |
| 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 |
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