ID:HEM6_HUMAN DESCRIPTION: RecName: Full=Coproporphyrinogen-III oxidase, mitochondrial; Short=COX; Short=Coprogen oxidase; Short=Coproporphyrinogenase; EC=1.3.3.3; Flags: Precursor; FUNCTION: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. CATALYTIC ACTIVITY: Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O. PATHWAY: Porphyrin metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1. SUBUNIT: Homodimer. SUBCELLULAR LOCATION: Mitochondrion intermembrane space. DISEASE: 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. SIMILARITY: Belongs to the aerobic coproporphyrinogen-III oxidase family. SEQUENCE CAUTION: Sequence=BAA04033.1; Type=Erroneous initiation; WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/CPOX";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P36551
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Gene Ontology (GO) Annotations with Structured Vocabulary
Molecular Function: GO:0004109 coproporphyrinogen oxidase activity GO:0005212 structural constituent of eye lens GO:0016491 oxidoreductase activity GO:0042802 identical protein binding GO:0042803 protein homodimerization activity
Biological 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:0046685 response to arsenic-containing substance GO:0051597 response to methylmercury GO:0055114 oxidation-reduction process
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
