Coproporphyrinogen III oxidase
| Coprogen_oxidas | |||||||||
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coproporphyrinogen iii oxidase from leishmania major
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| Identifiers | |||||||||
| Symbol | Coprogen_oxidas | ||||||||
| Pfam | PF01218 | ||||||||
| InterPro | IPR001260 | ||||||||
| PROSITE | PDOC00783 | ||||||||
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| Available protein structures: | |
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| Pfam | structures |
| PDB | RCSB PDB; PDBe; PDBj |
| PDBsum | structure summary |
Coproporphyrinogen-III oxidase, mitochondrial (abbreviated as CPOX) is an enzyme that in humans is encoded by the CPOX gene. 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.
CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation. The activity of the CPOX enzyme, located in the , is measured in lymphocytes.
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