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銅轉(zhuǎn)運蛋白質(zhì)β鏈抗體
  • 產(chǎn)品貨號：
    BN40485R
  • 中文名稱：
    銅轉(zhuǎn)運蛋白質(zhì)β鏈抗體
  • 英文名稱：
    Rabbit anti-ATP7B Polyclonal antibody
  • 品牌：
    Biorigin

  • 貨號

    產(chǎn)品規(guī)格

    售價

    備注

  • BN40485R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Chicken,Pig) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA

  • BN40485R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human,Mouse,Rat(predicted:Chicken,Pig) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA

產(chǎn)品描述

英文名稱ATP7B
中文名稱銅轉(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.  
研究領(lǐng)域細(xì)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WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量161kDa
細(xì)胞定位細(xì)胞漿 細(xì)胞膜 線粒體
性    狀Liquid
濃    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human ATP7B:1351-1465/1465 <Cytoplasmic>
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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)品介紹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.

SWISS:
P35670

Gene ID:
540

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(xì)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(xì)胞膜上的酶，它的功能主要是維持細(xì)胞內(nèi)外的離子及滲透壓平衡、跨膜電化學(xué)和細(xì)胞的能量代謝.






















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