Coproporphyrinogen III Oxidase Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | P36551 |
| Other Accession | P36551 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 50152 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase |
| Epitope Specificity | 361-454/454 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the aerobic coproporphyrinogen-III oxidase family. |
| SUBUNIT | Homodimer. |
| DISEASE | Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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 copropophyrinogen III. Mutations in the gene encoding CPOX are the cause of coproporphyria, an autosomal dominant disease characterized by skin photosensitivity and neurological disturbances. Symptoms are often experienced as attacks, which include severe abdominal and nerve pain. People affected by coproporphyria overexcrete copropophyrinogen III in feces and urine and the enzymatic activity of CPOX is found to be approximately half that of normal, leading to a decrease in overall heme synthesis. There is no cure for coproporphyria, but preventative treatment to relieve symptoms usually involves dietary changes and avoidance of drugs and alcohol. |
| Gene ID | 1371 |
|---|---|
| Other Names | COX; CPO; CPX; HARPO; HCP; M100835; Rgsc835; cac; nct; HEM6_HUMAN; CPOX; Coprogen oxidase; Coproporphyrinogenase; 1.3.3.3; HEM6_MOUSE; HEM6_RAT; coproporphyrinogen oxidase; coproporphyrinogen oxidase (coproporphyria, harderoporphyria); coproporphyria; homozygous coproporphyria |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | CPOX (HGNC:2321) |
|---|---|
| Synonyms | CPO, CPX |
| Function | Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX and participates to the sixth step in the heme biosynthetic pathway. |
| Cellular Location | Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q3B7D0} |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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Cat# AP99708
















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