Coproporphyrinogen Oxidase Recombinant Protein | CPOX recombinant protein
Recombinant Human Coproporphyrinogen Oxidase
Sterile Filtered colorless solution.
Introduction: Coproporphyrinogen Oxidase (CPOX) which is localized to the internal membrane space of erythrocytes takes part in the 6th phase of heme biosynthesis. CPOX catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. Mutations in human CPOX gene forecast the clinical result of the disease, with either hepatic hereditary coproporphyria or hematological manifestations of erythropoietic harderoporphyria.
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009]
Uniprot Description
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: EC 1.3.3.3; Mitochondrial; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Oxidoreductase
Chromosomal Location of Human Ortholog: 3q12
Cellular Component: mitochondrion; mitochondrial inner membrane; cytoplasm; mitochondrial intermembrane space
Molecular Function: coproporphyrinogen oxidase activity; protein homodimerization activity; structural constituent of eye lens
Biological Process: response to arsenic; porphyrin metabolic process; response to methylmercury; response to lead ion; response to insecticide; protoporphyrinogen IX biosynthetic process; response to iron ion; heme biosynthetic process
Disease: Coproporphyria, Hereditary
Research Articles on CPOX
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Product Notes
The CPOX cpox (Catalog #AAA146261) is a Recombinant Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The amino acid sequence is listed below: MGSSHHH HHH SSGLVPRGSH MGSTSL GRPE EEEDELAHRC SSFMAPPVTD LGELRRRPGD MKTKMELLIL ETQAQVCQAL AQVDGGANFS VDRWERKEGG GGISCVLQDG CVFEKAGVSI SVVHGNLSEE AAKQMRSRGK VLKTKDGKLP FCAMGVSSVI HPKNPHAPTI HFNYRYFEVE EADGNKQWWF GGGCDLTPTY LNQEDAVHFH RTLKEACDQH GPDLYPKFKK WCDDYFFIAH RGERRGIGGI FFDDLDSPSK EEVFRFVQSC ARAVVPSYIP LVKKHCDDSF TPQEKLWQQL RRGRYVEFNL LYDRGTKFGL FTPGSRIESI LMSLPLTARW EYMHSPSENS KEAEILEVLR HPRDWVR. It is sometimes possible for the material contained within the vial of "Coproporphyrinogen Oxidase, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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