產品編號 yb-1718R
英文名稱 Anti-ATP7B抗體
中文名稱 銅轉運蛋白質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.
 Anti-ATP7B抗體
說 明 書 0.1ml  0.2ml  
研究領域 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 Human, Mouse, Rat, Chicken, Pig, 
產品應用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:50-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 161kDa
細胞定位 細胞漿 細胞膜 線粒體
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
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.
 
PubMed PubMed
產品介紹 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.

 
銅轉運蛋白質β鏈是銅轉運蛋白質家族中的一種，可調節細胞內銅離子水平的銅轉運P型三磷酸腺苷酶，ATP7B是生物體內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外的離子及滲透壓平衡、跨膜電化學和細胞的能量代謝.