Ett enzym som katalyserar tetrapolymeriseringen av monopyrrolen porfobilinogen till att ge hydroximetylbilanen preuroporfyrinogen (se uroporfyrinogener) i flera diskreta steg. Det är det tredje enzymet i åttaenzymsbiosyntesen av hem. Hos människor leder brist på detta enzym, kodat av HMBS- eller PBGD-genen, till en form av neurologisk porfyri, akut intermittent porfyri. EC 2.5.1.61.
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
Inga svenska synonymer finns.
Synthase, Hydroxymethylbilane — Porphobilinogen Ammonia-Lyase — Ammonia-Lyase, Porphobilinogen — Porphobilinogen Ammonia Lyase — Porphobilinogen Deaminase — Deaminase, Porphobilinogen — Preuroporphyrinogen Synthetase — Synthetase, Preuroporphyrinogen — Uroporphyrinogen I Synthase — Synthase, Uroporphyrinogen I — Hydroxymethylbilane Synthetase — Synthetase, Hydroxymethylbilane — Pre-uroporphyrinogen Synthetase — Pre uroporphyrinogen Synthetase — Synthetase, Pre-uroporphyrinogen