Function and Biology

Uroporphyrinogen Decarboxylase single mutant D86E in complex with coproporphyrinogen-I

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

EC 4.1.1.37: Uroporphyrinogen decarboxylase

Reaction catalysed:
Uroporphyrinogen III = coproporphyrinogen + 4 CO(2)
Systematic name:
Uroporphyrinogen-III carboxy-lyase (coproporphyrinogen-III-forming)
Alternative Name(s):
  • Porphyrinogen carboxy-lyase
  • Porphyrinogen decarboxylase
  • Uroporphyrinogen III decarboxylase
  • Uroporphyrinogen-III carboxy-lyase

Sequence family

Pfam Protein family (Pfam)
PF01208
Domain description: Uroporphyrinogen decarboxylase (URO-D)
Occurring in:
  1. Uroporphyrinogen decarboxylase
The deposited structure of PDB entry 1r3v contains 1 copy of Pfam domain PF01208 (Uroporphyrinogen decarboxylase (URO-D)) in Uroporphyrinogen decarboxylase. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR006361
Domain description: Uroporphyrinogen decarboxylase HemE
Occurring in:
  1. Uroporphyrinogen decarboxylase
IPR000257
Domain description: Uroporphyrinogen decarboxylase (URO-D)
Occurring in:
  1. Uroporphyrinogen decarboxylase
IPR038071
Domain description: UROD/MetE-like superfamily
Occurring in:
  1. Uroporphyrinogen decarboxylase

Structure domain

CATH CATH domain
3.20.20.210
Class: Alpha Beta
Architecture: Alpha-Beta Barrel
Topology: TIM Barrel
Homology: TIM Barrel
Occurring in:
  1. Uroporphyrinogen decarboxylase
The deposited structure of PDB entry 1r3v contains 1 copy of CATH domain 3.20.20.210 (TIM Barrel) in Uroporphyrinogen decarboxylase. Showing 1 copy in chain A.
SCOP SCOP annotation
51727
Class: Alpha and beta proteins (a/b)
Fold: TIM beta/alpha-barrel
Superfamily: UROD/MetE-like
Occurring in:
  1. Uroporphyrinogen decarboxylase
The deposited structure of PDB entry 1r3v contains 1 copy of SCOP domain 51727 (Uroporphyrinogen decarboxylase, UROD) in Uroporphyrinogen decarboxylase. Showing 1 copy in chain A.