Function and Biology

Human porphobilinogen deaminase in complex with DPM cofactor

Source organism: Homo sapiens
Biochemical function: transferase activity
Biological process: heme O biosynthetic process
Cellular component: cytosol

EC 2.5.1.61: Hydroxymethylbilane synthase

Reaction catalysed:
4 porphobilinogen + H(2)O = hydroxymethylbilane + 4 NH(3)
Systematic name:
Porphobilinogen:(4-(2-carboxyethyl)-3-(carboxymethyl)pyrrol-2-yl)methyltransferase (hydrolyzing)
Alternative Name(s):
  • (4-(2-carboxyethyl)-3-(carboxymethyl)pyrrol-2-yl)methyltransferase (hydrolyzing)
  • HMB-synthase
  • Porphobilinogen ammonia-lyase (polymerizing)
  • Porphobilinogen deaminase
  • Pre-uroporphyrinogen synthase
  • Uroporphyrinogen I synthase
  • Uroporphyrinogen I synthetase
  • Uroporphyrinogen synthase
  • Uroporphyrinogen synthetase

Sequence families

Pfam Protein families (Pfam)
PF03900
Domain description: Porphobilinogen deaminase, C-terminal domain
Occurring in:
  1. Porphobilinogen deaminase
The deposited structure of PDB entry 5m7f contains 2 copies of Pfam domain PF03900 (Porphobilinogen deaminase, C-terminal domain) in Porphobilinogen deaminase. Showing 1 copy in chain A.

PF01379
Domain description: Porphobilinogen deaminase, dipyromethane cofactor binding domain
Occurring in:
  1. Porphobilinogen deaminase
The deposited structure of PDB entry 5m7f contains 2 copies of Pfam domain PF01379 (Porphobilinogen deaminase, dipyromethane cofactor binding domain) in Porphobilinogen deaminase. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR000860
Domain description: Porphobilinogen deaminase
Occurring in:
  1. Porphobilinogen deaminase
IPR036803
Domain description: Porphobilinogen deaminase, C-terminal domain superfamily
Occurring in:
  1. Porphobilinogen deaminase
IPR022419
Domain description: Porphobilinogen deaminase, dipyrromethane cofactor binding site
Occurring in:
  1. Porphobilinogen deaminase
IPR022418
Domain description: Porphobilinogen deaminase, C-terminal
Occurring in:
  1. Porphobilinogen deaminase
IPR022417
Domain description: Porphobilinogen deaminase, N-terminal
Occurring in:
  1. Porphobilinogen deaminase

Structure domains

CATH CATH domains
3.40.190.10
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: D-Maltodextrin-Binding Protein; domain 2
Homology: Periplasmic binding protein-like II
Occurring in:
  1. Porphobilinogen deaminase
The deposited structure of PDB entry 5m7f contains 4 copies of CATH domain 3.40.190.10 (D-Maltodextrin-Binding Protein; domain 2) in Porphobilinogen deaminase. Showing 2 copies in chain A.
3.30.160.40
Class: Alpha Beta
Architecture: 2-Layer Sandwich
Topology: Double Stranded RNA Binding Domain
Homology: Porphobilinogen deaminase, C-terminal domain
Occurring in:
  1. Porphobilinogen deaminase
The deposited structure of PDB entry 5m7f contains 2 copies of CATH domain 3.30.160.40 (Double Stranded RNA Binding Domain) in Porphobilinogen deaminase. Showing 1 copy in chain A.