hydroxymethylbilane synthase
Sign in to saveAlso known as uroporphyrinogen I synthase, porphyria, acute; Chester type, porphobilinogen deaminase, pre-uroporphyrinogen synthase, uroporphyrinogen I synthetase, HMBS
mammalian protein found in Homo sapiens
Protein · UniProt
Porphobilinogen deaminase
- Gene
- HMBS
- Organism
- Homo sapiens (Human)
- Length
- 361 aa
- Molecular mass
- 39,330 Da
- Evidence
- 1: Evidence at protein level
As part of the heme biosynthetic pathway, catalyzes the sequential polymerization of four molecules of porphobilinogen to form hydroxymethylbilane, also known as preuroporphyrinogen (PubMed:18004775, PubMed:18936296, PubMed:19138865, PubMed:23815679). Catalysis begins with the assembly of the dipyrromethane cofactor by the apoenzyme from two molecules of porphobilinogen or from preuroporphyrinogen. The covalently linked cofactor acts as a primer, around which the tetrapyrrole product is assembled (PubMed:18936296). In the last step of catalysis, the product, preuroporphyrinogen, is released…
Swiss-Prot (reviewed) · via UniProt
Clinical Trials
1 registered- PHASE2/PHASE3COMPLETEDPorphozym in the Treatment of Acute Attacks in AIPZymenex A/S · NCT00418795