CPOX, Official Symbol CPOXprovided by HGNC Official Full Name coproporphyrinogen oxidaseprovided by HGNC Primary source HGNC:HGNC:2321 See related Ensembl:ENSG00000080819 MIM:612732; AllianceGenome:HGNC:2321 Gene type protein coding RefSeq status REVIEWED Organism Homo sapiens Lineage Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo Also known as COX; CPO; CPX; HCP; HARPO Summary 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).[provided by RefSeq, Oct 2009] Expression Ubiquitous expression in bone marrow (RPKM 5.0), liver (RPKM 3.1) and 24 other tissues See more Orthologs mouse all