CPOX, Recombinant Protein

Coproporphyrinogen-III oxidase mitochondrial precursor; Coproporphyrinogen-III oxidase; mitochondrial precursor; CPO; CPX; HCP

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

E Coli

> 95% by SDS-PAGE

Liquid. In 20mM Tris-HCl Buffer (pH 8.0) containing 0.15M NaCl, 10% glycerol, 1mM DTT

1 mg/ml (determined by Bradford assay) (lot specific)

Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.

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.

Coproporphyrinogen-III oxidase, mitochondrial precursor, also known as CPOX, is a 454 amino acid mitochondrial enzyme that is localized to the inner membrane space of erythrocytes. It participates in the sixth step of heme biosynthesis by catalyzing the formation of protoporphyrinogen IX from coproporphyrinogen III. Mutations in human CPOX gene predict the clinical outcome of the disease, with either hepatic hereditary coproporphyria or hematological manifestations of erythropoietic harderoporphyria. Recombinant human CPOX protein, fused to His-tag at N-terminus, was expressed in E Coli and purified by using conventional chromatography techniques.

Metabolism

SDS-PAGE

41.6 kDa (367aa) confirmed by MALDI-TOF

coproporphyrinogen oxidase

oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

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

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]

Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.

Martasek P., et al. (1994) Hum Mol Genet. 3:477-480; Lamoril J., et al. (1995) Hum Mol Genet. 4:275-278.;

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