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500ug
| 产品编号 | bs-13980P |
| 英文名称 | CPOX Antibody Blocking Peptide |
| 中文名称 | 粪生素氧化酶CPOX封闭多肽 |
| 英文别名 | Coproporphyrinogen Oxidase; Coprogen oxidase; COPROPORPHYRIA; Coproporphyrinogen III oxidase; Coproporphyrinogen-III oxidase; Coproporphyrinogenase; COX; CPO; CPOX; CPX; HEM 6; Hem-6; Hem6; HEM6_HUMAN; mitochondrial. |
| 纯化方法 | HPLC |
| 研究领域 | Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers Signal Transduction > Metabolism > Mitochondrial |
| 亚基 | Homodimer. |
| 亚细胞定位 | Mitochondrion intermembrane space. |
| 相似性 | Belongs to the aerobic coproporphyrinogen-III oxidase family. |
| 功能 | Coproporphyrinogen Oxidase (CPOX) is the sixth enzyme of the heme biosynthetic pathway. This soluble protein is localized in the intermembrane space of mitochondria and its catalytic activy mediates the coversion of Coproporphyrinogen-III + O2 + 2 H+ into Protoporphyrinogen-IX + 2 CO2 + 2 H2O. Defects in CPOX are the cause of hereditary coproporphyria |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
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CPOX Antibody Blocking Peptide(bs-13980P)-500ug
¥880







