Enzymes Recombinant Proteins

Human CPOX Recombinant Protein (RPPB1505)

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SKU:
RPPB1505
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P36551
Research Area:
Enzymes

Description

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Product Name:Human CPOX Recombinant Protein
Product Code:RPPB1505
Size:20µg
Species:Human
Target:CPOX
Synonyms:CPO, CPX, HCP, Coproporphyrinogen-III oxidase, mitochondrial, COX, Coprogen oxidase, Coproporphyrinogenase, CPOX.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The CPOX solution (1mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 10% glycerol and 1mM DTT.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 95% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSTSLGRPE 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

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.

CPOX Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 367 amino acids (111-454) 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.

UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Oxidoreductase; EC 1.3.3.3; Mitochondrial; Cofactor and Vitamin Metabolism - porphyrin and chlorophyll

Chromosomal Location of Human Ortholog: 3q12

Cellular Component: mitochondrion; cytoplasm; mitochondrial inner membrane; mitochondrial intermembrane space

Molecular Function:coproporphyrinogen oxidase activity; protein homodimerization activity; structural constituent of eye lens

Biological Process: response to arsenic; response to methylmercury; response to lead ion; porphyrin metabolic process; response to insecticide; protoporphyrinogen IX biosynthetic process; response to iron ion; heme biosynthetic process

Disease: Coproporphyria, Hereditary

NCBI Summary: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 Code:P36551
NCBI GenInfo Identifier:67476671
NCBI Gene ID:1371
NCBI Accession:P36551.3
UniProt Related Accession:P36551
Molecular Weight:36kDa
NCBI Full Name:Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
NCBI Synonym Full Names:coproporphyrinogen oxidase
NCBI Official Symbol:CPOX��
NCBI Official Synonym Symbols:COX; CPO; CPX; HCP��
NCBI Protein Information:oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
UniProt Protein Name:Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
Protein Family:Oxygen-dependent coproporphyrinogen-III oxidase
UniProt Gene Name:CPOX��
UniProt Entry Name:HEM6_HUMAN
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