Coproporphyrinogen III oxidase

Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

Coprogen oxidase
Coprogen oxidase
	coproporphyrinogen iii oxidase from leishmania major
Identifiers
Symbol	Coprogen oxidase
Pfam	PF01218
InterPro	IPR001260
PROSITE	PDOC00783
Pfam
Available protein structures:
Pfam	structures / ECOD
PDB	RCSB PDB; PDBe; PDBj
PDBsum	structure summary

CPOX
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	2AEX
Identifiers
Aliases	CPOX, CPO, CPX, HCP, coproporphyrinogen oxidase, COX, HARPO
External IDs	OMIM: 612732; MGI: 104841; HomoloGene: 76; GeneCards: CPOX; OMA:CPOX - orthologs
EC number	1.3.3.3
Gene location (Mouse)
Chr.	Chromosome 16 (mouse)
End	58,537,999 bp
RNA expression pattern
	Top expressed in
	trabecular bone; ; jejunal mucosa; ; corpus callosum; ; right lobe of liver; ; bone marrow; ; inferior ganglion of vagus nerve; ; bone marrow cells; ; rectum; ; monocyte; ; duodenum;
	Top expressed in
	human fetus; ; atrium; ; tibiofemoral joint; ; fetal liver hematopoietic progenitor cell; ; yolk sac; ; epithelium of small intestine; ; right kidney; ; pontine nuclei; ; ventral tegmental area; ; habenula;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	coproporphyrinogen oxidase activity; protein homodimerization activity; structural constituent of eye lens; identical protein binding; oxidoreductase activity;
Cellular component	membrane; mitochondrial intermembrane space; mitochondrion; mitochondrial inner membrane; cytosol; cytoplasm;
Biological process	response to arsenic-containing substance; protoporphyrinogen IX biosynthetic process; porphyrin-containing compound biosynthetic process; response to iron ion; heme biosynthetic process; response to lead ion; response to inorganic substance; response to insecticide; response to methylmercury; protoporphyrinogen IX biosynthetic process from glutamate;
	Sources:Amigo / QuickGO
Orthologs
	1371
	12892
	n/a
	ENSMUSG00000022742
	P36551
	P36552
	NM_000097
	NM_007757
	NP_000088
	NP_031783
	Wikidata
View/Edit Human	View/Edit Mouse

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 mitochondrial membrane, is measured in lymphocytes.