Coproporphyrinogen III oxidase
Mammalian protein found in Homo sapiens / From Wikipedia, the free encyclopedia
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Coproporphyrinogen-III oxidase, mitochondrial (abbreviated as CPOX) is an enzyme that in humans is encoded by the CPOX gene.[4][5][6] 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.[7][8]
Quick Facts CPOX, Available structures ...
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Quick Facts Coprogen oxidase, Identifiers ...
Coprogen oxidase | |||||||||
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Identifiers | |||||||||
Symbol | Coprogen oxidase | ||||||||
Pfam | PF01218 | ||||||||
InterPro | IPR001260 | ||||||||
PROSITE | PDOC00783 | ||||||||
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CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation.[9] The activity of the CPOX enzyme, located in the mitochondrial membrane, is measured in lymphocytes.[10]