NP_001032172.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
Molecular Weight
49,278 Da
NCBI Official Full Name
oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
NCBI Official Synonym Full Names
coproporphyrinogen oxidase
NCBI Protein Information
oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
UniProt Protein Name
Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
UniProt Synonym Gene Names
UniProt Entry Name
HEM6_RAT
UniProt Comments for CPOX
CPOX: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. Defects in CPOX are the cause of hereditary coproporphyria (HCP). 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. Belongs to the aerobic coproporphyrinogen-III oxidase family.
Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; EC 1.3.3.3; Mitochondrial; Oxidoreductase
Cellular Component: mitochondrion; cytoplasm; mitochondrial inner membrane; mitochondrial intermembrane space
Molecular Function: coproporphyrinogen oxidase activity; identical protein binding; protein homodimerization activity; structural constituent of eye lens
Biological Process: response to inorganic substance; response to arsenic; response to methylmercury; response to lead ion; response to insecticide; response to iron ion; protoporphyrinogen IX biosynthetic process; heme biosynthetic process
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Products associated with CPOX sirna
Pathways associated with CPOX sirna
Diseases associated with CPOX sirna
Organs/Tissues associated with CPOX sirna
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