| 產(chǎn)品編號 | bs-1718R-RBITC |
| 英文名稱 | Rabbit Anti-ATP7B/RBITC Conjugated antibody |
| 中文名稱 | 羅丹明（RBITC）標(biāo)記的銅轉(zhuǎn)運蛋白質(zhì)β鏈抗體 |
| 別 名 | ATPase Cu++ transporting beta polypeptide; Copper pump 2; Copper transporting ATPase 2; PWD; Toxic milk; tx; WC1; Wilson disease associated protein; WND. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 細胞生物 激酶和磷酸酶 線粒體 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse, Rat, (predicted: Human, Chicken, Pig, ) |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 161kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ATP7B |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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. |
| 產(chǎn)品介紹 |
background: ATP7b is an important protein for copper transport and elimination of excess copper from the body. ATP7b transports metals in and out of cells using ATP. There are 3 known isoforms of the ATP7b gene; A is found in the liver, kidney, and brain, the shorter form B is found in brain tissue, and the third isoform, known as WND/140 KDA is found in mitochondria. Mutations in the ATP7b gene can cause Wilson's disease, an inherited disorder causing copper poisoning in the brain and liver. Function: Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile. Subunit: Monomer. Interacts with COMMD1/MURR1. Subcellular Location: Golgi apparatus, trans-Golgi network membrane; Multi-pass membrane protein. Isoform 2: Cytoplasm. WND/140 kDa: Mitochondrion. Tissue Specificity: Most abundant in liver and kidney and also found in brain. Isoform 2 is expressed in brain but not in liver. The cleaved form WND/140 kDa is found in liver cell lines and other tissues. Post-translational modifications: Isoform 1 may be proteolytically cleaved at the N-terminus to produce the WND/140 kDa form. DISEASE: Defects in ATP7B are the cause of Wilson disease (WD) [MIM:277900]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis. Similarity: Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily. Contains 6 HMA domains. Database links: Entrez Gene: 540 Human Omim: 606882 Human SwissProt: P35670 Human Unigene: 492280 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 銅轉(zhuǎn)運蛋白質(zhì)β鏈?zhǔn)倾~轉(zhuǎn)運蛋白質(zhì)家族中的一種，可調(diào)節(jié)細胞內(nèi)銅離子水平的銅轉(zhuǎn)運P型三磷酸腺苷酶，ATP7B是生物體內(nèi)廣泛存在的一種極為重要的細胞膜上的酶，它的功能主要是維持細胞內(nèi)外的離子及滲透壓平衡、跨膜電化學(xué)和細胞的能量代謝. |
