InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: CPOX

Summary

InnateDB Protein

IDBP-46914.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

CPOX

Protein Name

coproporphyrinogen oxidase

Synonyms

CPO; CPX; HCP;

Species

Homo sapiens

Ensembl Protein

ENSP00000264193

InnateDB Gene

IDBG-46912 (CPOX)

Protein Structure

UniProt Annotation

Function

Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.

Subcellular Localization

Mitochondrion intermembrane space.

Disease Associations

Hereditary coproporphyria (HCP) [MIM:121300]: A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. Hereditary coproporphyria is an acute hepatic porphyria characterized by skin photosensitivity, attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Hereditary coproporphyria is biochemically characterized by overexcretion of coproporphyrin III in the urine and in the feces. {ECO:0000269PubMed:12181641, ECO:0000269PubMed:15896662, ECO:0000269PubMed:16398658, ECO:0000269PubMed:7757079, ECO:0000269PubMed:7849704, ECO:0000269PubMed:8012360, ECO:0000269PubMed:8990017, ECO:0000269PubMed:9048920, ECO:0000269PubMed:9298818, ECO:0000269PubMed:9888388}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 6 experimentally validated interaction(s) in this database.

Experimentally validated
Total	6 [view]
Protein-Protein	6 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004109	coproporphyrinogen oxidase activity
GO:0005212	structural constituent of eye lens
GO:0042802	identical protein binding
GO:0042803	protein homodimerization activity

Biological Process

GO:0006778	porphyrin-containing compound metabolic process
GO:0006779	porphyrin-containing compound biosynthetic process
GO:0006782	protoporphyrinogen IX biosynthetic process
GO:0006783	heme biosynthetic process
GO:0010035	response to inorganic substance
GO:0010039	response to iron ion
GO:0010288	response to lead ion
GO:0017085	response to insecticide
GO:0044281	small molecule metabolic process
GO:0046685	response to arsenic-containing substance
GO:0051597	response to methylmercury
GO:0055114	oxidation-reduction process