4 PBGs bind to form HMB
4 PBG + H2O → HMBL + 4 NH4+
- Reactome
- R-HSA-189406
- Compartment
- cytosol
- Rhea
- RHEA:13186
Inputs
- 4 PBGCHEBI:58126, C10H13N2O4, cytosol
- H2OCHEBI:15377, H2O, cytosol
Outputs
- HMBLCHEBI:57845, C40H38N4O17, cytosol
- 4 NH4+CHEBI:28938, H4N, cytosol
Catalysed by
- hydroxymethylbilane synthase activity of HMBS:DIPY [cytosol]EC 2.5.1.61
Literature
- Human porphobilinogen deaminase mutations in the investigation of the mechanism of dipyrromethane cofactor assembly and tetrapyrrole formation Biochem Soc Trans, 2003
- Purification and properties of uroporphyrinogen I synthase from human erythrocytes. Identification of stable enzyme-substrate intermediates. J Biol Chem, 1980