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Coproporphyrinogen III oxidase : ウィキペディア英語版 | Coproporphyrinogen III oxidase
Coproporphyrinogen-III oxidase, mitochondrial is an enzyme that in humans is encoded by the ''CPOX'' gene.〔(【引用サイトリンク】 url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=1371 )〕 A genetic defect in the enzyme results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria.〔(【引用サイトリンク】url=http://ghr.nlm.nih.gov/gene/CPOX )〕 It is an enzyme involved in the sixth step of porphyrin metabolism it catalyses the oxidative decarboxylation of coproporphyrinogen III to proto-porphyrinogen IX in the haem and chlorophyll biosynthetic pathways.〔 The protein is a homodimer containing two internally bound iron atoms per molecule of native protein. The enzyme is active in the presence of molecular oxygen that acts as an electron acceptor. The enzyme is widely distributed having been found in a variety of eukaryotic and prokaryotic sources. == References ==
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