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uroporphyrinogen decarboxylase; UPD (UROD)
Function:
- catalyzes decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III
- porphyrin metabolism; protoporphyrin-IX biosynthesis
- coproporphyrinogen-III from 5-aminolevulinate: step 4/4
uroporphyrinogen III = coproporphyrinogen + 4 CO2
Structure:
- homodimer
- belongs to the uroporphyrinogen decarboxylase family
Compartment: cytoplasm
Pathology:
- defects in UROD are the cause of
a) familial porphyria cutanea tarda
b) hepatoerythropoietic porphyria
Interactions
molecular events
Related
erythropoietic protoporphyria (EPP)
heme synthesis
porphyria cutanea tarda (PCT)
General
carboxy-lyase or decarboxylase
phosphoprotein
Properties
SIZE: entity length = 367 aa
MW = 41 kD
COMPARTMENT: cytoplasm
MOTIF: acetylation site
SITE: N-TERMINUS
EFFECTOR-BOUND: acetyl
Substrate binding {37-41}
binding site
SITE: 55-55
FOR-BINDING-OF: Substrate
Ser phosphorylation site {S61}
binding site
SITE: 85-85
FOR-BINDING-OF: Substrate
binding site
SITE: 86-86
FOR-BINDING-OF: Substrate
MOTIF: aspartate residue {86}
binding site
SITE: 164-164
FOR-BINDING-OF: Substrate
binding site
SITE: 219-219
FOR-BINDING-OF: Substrate
binding site
SITE: 339-339
FOR-BINDING-OF: Substrate
Database Correlations
OMIM correlations
MORBIDMAP 613521
UniProt P06132
Pfam PF01208
Entrez Gene 7389
Kegg hsa:7389
ENZYME 4.1.1.37
References
- UniProt :accession P06132
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/UROD
- Wikipedia; Note: uroporphyrinogen III decarboxylase entry
http://en.wikipedia.org/wiki/uroporphyrinogen_III_decarboxylase