精品国产av自拍_精品日韩欧美在线视频一区二区_一区二区在线观看在线_福利在线观看免费高清完整版_无码免费动漫老黄网站_无码一区在线观看视频_精品五月精品婷婷_免费国产日本高清_亚洲特黄特色一级在线观看_国产Ⅴ亚洲Ⅴ欧美Ⅴ专区

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
内射干少妇亚洲69XXX,国产午夜福利100集发布,久久91精品国产91久
Rabbit Anti-ATP7B/Biotin Conjugated antibody (bs-1718R-Bio)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-1718R-Bio
英文名稱 Rabbit Anti-ATP7B/Biotin Conjugated antibody
中文名稱 生物素標(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 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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.
版權(quán)所有 2004-2026 0592123.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
国产精品看片在线观看 | 免费黄色网站在线观看 | 洗澡被公强奷30分钟在线观看 | 亚洲日韩AV一级午夜在线 | 四虎影视成人永久免费观看视频 | 国产精品福利在线观看免费不卡 | 精品国产乱码欠欠欠欠精品 | 动漫黄网站免费永久在线观看 | 人妻互换免费中文字幕 | 久久久久久久成人午夜精品福利 | 亚洲国产综合久久久精品 | 一级毛片丰满**出奶水 | 少妇伦子伦情品无吗 | 欧美群交在线播放1 | 邻居少妇张开腿让我爽了在线观看 | 久久精品国产99久久99久久久 | 亚洲精品无码天堂AV | 久色乳综合思思在线视频 | 成人无码片黄网站A毛片免费 | 亚洲精品无码高潮喷水 | 国产男人午夜视频在线观看 | 加勒比AV一本大道香蕉大在线 | 日本免费在线观看视频 | 久久久久国产精品夜夜夜夜夜 | 打扑克视频全程不盖被子打扑克 | 品产品久精国精产拍20 | 在线观看欧美亚洲日韩片 | 亚洲国产18成人中文字幕久久久久无码av | 一本久久a久久精品亚洲 | 国产精品亚洲精品专区 | 国产亚洲一区在线 | 2018国产精华国产精品 | 欧美两根一起进3p在线观看 | 国产AV一区二区高潮久久 | 精人妻无码一区二区三区 | 91精品国产福利资源在线观看麻豆 | 在线观着免费观看国产黄 | 99久久精品费精品国产一区二区 | 内射人妻无码色AV无码 | 国产精品你当然比游戏重要 | 中国女人一级毛片 |