Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

coproporphyrinogen oxidase

Name from literature

coproporphyrinogen oxidase

Pathway from literature

heme synthesis

Pathway from KEGG

Metabolism of Cofactors and Vitamins; Porphyrin and chlorophyll metabolism; map00860


Human (9606)

Genome localization

3q12[1371 ],

Rate-limiting Description

"During neuronal differentiation, there were increases in the cellular heme levels and increases in the mRNA levels for the rate-limiting enzymes of heme biosynthesis, such as aminolevulinic acid synthase (ALAS; EC and coproporphyrinogen oxidase (EC" (16567402)

"Ethanol suppresses the activity of porphobilinogen synthase (synonym: delta-aminolevulinic acid dehydratase), uroporphyrinogen decarboxylase, coproporphyrinogen oxidase and ferrochelatase, whereas it induces the first and rate-limiting enzyme in the pathway, delta-aminolevulinic acid synthase and also porphobilinogen deaminase." (10787385)

Regulatory Information

Upstream transcription factor


Regulatory type


transcriptional factor;CPRE binding protein

"Coproporphyrinogen oxidase [CPO] gene promoter regulatory element (CPRE) plays an important role in CPO gene regulation." (10873651#9787185)

transcriptional factor;K562 cells-derived leucine zipper-like protein 1(57106)

"Klp1 (K562 cells-derived leucine zipper-like protein 1) is a transcription factor which binds to the coproporphyrinogen oxidase promoter regulatory element (GGACTACAG)." (11779635)

Gene ontology

Gene ontology

GO:0006783 (P) heme biosynthetic process [P36551 ];
GO:0005758 (C) mitochondrial intermembrane space [P36551 ];
GO:0004109 (F) coproporphyrinogen oxidase activity [P36551 ];
GO:0055114 (P) oxidation reduction [P36551 ];

Subcellular localization


mitochondrion [P36551 ];

Disease relevance


Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase activity [P36551 ];




Entrez Gene




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  Last Modified: 2009-03-24  
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