Coproporphyrinogen Oxidase, Recombinant Protein

CPOX Human; Coproporphyrinogen Oxidase Human Recombinant; CPO; CPX; HCP; Coproporphyrinogen-III oxidase; mitochondrial; COX; Coprogen oxidase; Coproporphyrinogenase; CPOX

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 95% as determined by SDS-PAGE.

The CPOX solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 10% glycerol and 1mM DTT.
Sterile Filtered colorless solution.

Small volumes of CPOX recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: CPOX Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 367 amino acids (114-454) and having a molecular mass of 41.6kDa. CPOX is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

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.

ENZYMES; Enzymes; Oxidase

30,383 Da

coproporphyrinogen oxidase

oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial; COX; coprogen oxidase; coproporphyrinogenase

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

CPO; CPX; COX; Coprogen oxidase; Coproporphyrinogenase  [Similar Products]

HEM6_HUMAN

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]

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

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