Peptide sequence: EVFRFVQSCA RAVVPSYIPL VKKHCDDSFT PQEKLWQQLR RGRYVEFNLL
The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).
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Protein Aliases: coprogen oxidase; coproporphyrinogen-III oxidase, mitochondrial; coproporphyrinogenase; COX; Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
Gene Aliases: CPO; CPOX; CPX; HCP
UniProt ID: (Human) P36551
Entrez Gene ID: (Human) 1371