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Coproporphyrinogen-III oxidase, mitochondrial; COX; Coprogen oxidase; Coproporphyrinogenase (CPOX, CPO, CPX)
Function:
- key enzyme in heme biosynthesis
- catalyzes oxidative decarboxylation of propionic acid side chains of rings A & B of coproporphyrinogen III
- porphyrin metabolism; protoporphyrin-IX biosynthesis
- protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1
Coproporphyrinogen-III + O2 + 2 H+ = protoporphyrinogen-IX + 2 CO2 + 2 H2O
Structure:
- homodimer
- belongs to the aerobic coproporphyrinogen-III oxidase family
Compartment: mitochondria intermembrane space
Pathology:
- defects in CPOX are the cause of hereditary coproporphyria
Interactions
molecular events
Related
heme synthesis
General
membrane protein
mitochondrial protein
oxidoreductase
Properties
SIZE: entity length = 454 aa
MW = 50 kD
COMPARTMENT: mitochondria
MOTIF: domain {193-202}
binding site
SITE: 244-244
FOR-BINDING-OF: Substrate
histidine residue {H258}
Substrate binding {260-262}
histidine residue {327}
domain {392-428}
MOTIF: Substrate binding {411-416}
Database Correlations
OMIM correlations
UniProt P36551
Pfam PF01218
Entrez Gene 1371
Kegg hsa:1371
ENZYME 1.3.3.3
References
- UniProt :accession P36551
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/CPOX