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

More general terms

• oxidoreductase
• membrane protein
• mitochondrial protein

Additional terms

• heme synthesis

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=1371
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:1371
• OMIM: https://mirror.omim.org/entry/121300
• OMIM: https://mirror.omim.org/entry/612732
• UniProt: http://www.uniprot.org/uniprot/P36551.html