本公司经销ATP7B，铜转运蛋白质β链抗体，克隆类型为polyclonal，宿主来源是Rabbit，ATP7B铜转运蛋白质β链抗体可应用于WB、elisa、IP、IF、IHC等实验，欢迎垂询订购！

货号：BY-1718R
英文名称：Anti-ATP7B
中文名称：铜转运蛋白质β链抗体
其他名称：ATPase Cu++ transporting beta polypeptide; Copper pump 2; Copper transporting ATPase 2; PWD; Toxic milk; tx; WC1; Wilson disease associated protein; WND.
抗体来源：Rabbit
克隆类型：polyclonal
蛋白分子量：predicted molecular weight: 161kDa
纯化方法：affinity purified by Protein A
交叉反应：hu, mo, rat, pig, chk
产品介绍：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.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.