| 产品编号 | bs-8860R |
| 英文名称 | Coproporphyrinogen III Oxidase Rabbit pAb |
| 中文名称 | 原卟啉氧化酶3抗体 |
| 别 名 | Coprogen oxidase; COPROPORPHYRIA; Coproporphyrinogen III oxidase; Coproporphyrinogenase; COX; CPO; CPOX; CPX; HEM 6; Hem-6; Hem6; HEM6_HUMAN. |
| 研究领域 | 细胞生物 发育生物学 干细胞 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 克 隆 号 | |
| 交叉反应 | (predicted: Human,Mouse,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Dog) |
| 产品应用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 39 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞浆 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CPOX/Coproporphyrinogen Oxidase: 361-454/454 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store 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. |
| PubMed | PubMed |
| 产品介绍 |
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. Function: 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 Subunit: Homodimer. Subcellular Location: Mitochondrion intermembrane space. 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. Similarity: Belongs to the aerobic coproporphyrinogen-III oxidase family. SWISS: P36551 Gene ID: 1371 Database links: Entrez Gene: 1371 Human Entrez Gene: 12892 Mouse Omim: 612732 Human SwissProt: P36551 Human SwissProt: P36552 Mouse Unigene: 476982 Human Unigene: 291519 Mouse Unigene: 19581 Rat |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
