FITC标记的铜转运蛋白质β链抗体
产品名称： FITC标记的铜转运蛋白质β链抗体
英文名称： Anti-ATP7B/FITC
产品编号： HZ-1718R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-ATP7B/FITC Conjugated antibody
FITC标记的铜转运蛋白质β链抗体
| 英文名称Anti-ATP7B/FITC中文名称FITC标记的铜转运蛋白质β链抗体别 名ATPase Cu++ transporting beta polypeptide; Copper pump 2; Copper transporting ATPase 2; PWD; Toxic milk; tx; WC1; Wilson disease associated protein; WND. 规格价格100ul/2980元 购买 大包装/询价说 明 书100ul 研究领域细胞生物 激酶和磷酸酶 线粒体 抗体来源Rabbit克隆类型Polyclonal交叉反应Human, Mouse, Rat, Chicken, Pig, 产品应用IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.分 子 量161kDa细胞定位细胞膜 线粒体性 状Lyophilized or Liquid浓 度1mg/ml免 疫 原KLH conjugated synthetic peptide derived from human ATP7B亚 型IgG纯化方法affinity purified by Protein A储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.保存条件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.产品介绍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. 铜转运蛋白质β链是铜转运蛋白质家族中的一种，可调节细胞内铜离子水平的铜转运P型三磷酸腺苷酶，ATP7B是生物体内广泛存在的一种极为重要的细胞膜上的酶，它的功能主要是维持细胞内外的离子及渗透压平衡、跨膜电化学和细胞的能量代谢. |
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ATP7B是铜转运和清除体内多余铜的重要蛋白质。ATP7B用ATP运送细胞进出细胞。ATP7b基因有三种已知亚型：A在肝脏、肾脏和脑中发现，B在脑组织中发现较短的亚型，第三种亚型WND/140KDA在线粒体中发现。ATP7B基因的突变可引起Wilson病，这是遗传性疾病，导致大脑和肝脏中的铜中毒。