| 產(chǎn)品編號 | bs-13980R |
| 英文名稱 | Rabbit Anti-CPOX antibody |
| 中文名稱 | 糞生素氧化酶CPOX抗體 |
| 別 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. |
| 研究領(lǐng)域 | 腫瘤 細胞生物 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo) 新陳代謝 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Rabbit,Sheep,Chicken,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 39kDa |
| 細胞定位 | 細胞漿 線粒體 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Coproporphyrinogen Oxidase: 351-454/454 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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] Function: Key enzyme in heme biosynthesis. Catalyzes the oxidative decarboxylation of propionic acid side chains of rings A and B of coproporphyrinogen III. 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 |
