亚洲制服欧美另类-午夜激情av电影-日本高清中文字幕一区二区三区-中国欧美日韩一区二区三区-欧洲亚洲日本韩国-成人欧美激情一区二区-亚洲偷偷自拍高清

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯(lián)系我們
香蕉有码在线视频发布,韩日AV无码片一区二区三区
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-WFS1/PE-Cy5.5 Conjugated antibody (bs-11272R-PE-Cy5.5)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-11272R-PE-Cy5.5
英文名稱 Rabbit Anti-WFS1/PE-Cy5.5 Conjugated antibody
中文名稱 PE-Cy5.5標記的Wolfram綜合征蛋白1抗體
別    名 DFNA14; DFNA38; DFNA6; DIDMOAD; WFRS; WFS; Wolfram syndrome 1 (wolframin); Wolfram syndrome; WOLFRAMIN; WFS1_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  神經生物學  信號轉導  生長因子和激素  糖尿病  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Cow, Horse, )
產品應用 ICC=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 97kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human WFS1
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.
產品介紹 background:
Wolfram syndrome protein (WFS1) is an 890 amino acid protein that contains a cytoplasmic N-terminal domain, followed by nine-transmembrane domains and a luminal C-terminal domain. WFS1 is predominantly localized to the endoplasmic reticulum (ER) (1) and its expression is induced in response to ER stress, partially through transcriptional activation (2,3). Research studies have shown that mutations in the WFS1 gene lead to Wolfram syndrome, an autosomal recessive neurodegenerative disorder defined by young-onset, non-immune, insulin-dependent diabetes mellitus and progressive optic atrophy (4).

Function:
WFS1 is a novel component of Wolfram syndrome, a rare form of juvenile diabetes. WFS1 plays an important role in maintaining homeostasis of the endoplasmic reticulum (ER) in the pancreas. It is normally up-regulated during insulin secretion, whereas inactivation of the protein can cause ER stress. Chronic ER stress is a major involvement in Wolfram syndrome.

Subcellular Location:
Endoplasmic reticulum; endoplasmic reticulum membrane; multipass membrane protein

Tissue Specificity:
Highly expressed in heart followed by brain, placenta, lung and pancreas. Weakly expressed in liver, kidney and skeletal muscle. Also expressed in islet and beta-cell insulinoma cell line.

DISEASE:
Defects in WFS1 are the cause of Wolfram syndrome type 1 (WFS1) [MIM:222300]. A rare autosomal recessive disorder characterized by juvenile diabetes mellitus, diabetes insipidus, optic atrophy, deafness and various neurological symptoms.
Defects in WFS1 are the cause of deafness autosomal dominant type 6 (DFNA6) [MIM:600965]; also called non-syndromic sensorineural deafness autosomal dominant type 14 (DFNA14) or non-syndromic sensorineural deafness autosomal dominant type 38 (DFNA38). DFNA6 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. DFNA6 is a low-frequency hearing loss in which frequencies of 2000 Hz and below are predominantly affected. Many patients have tinnitus, but there are otherwise no associated features such as vertigo. Because high-frequency hearing is generally preserved, patients retain excellent understanding of speech, although presbycusis or noise exposure may cause high-frequency loss later in life. DFNA6 worsens over time without progressing to profound deafness.
Defects in WFS1 are the cause of Wolfram-like syndrome autosomal dominant (WFSL) [MIM:614296]. A disease characterized by the clinical triad of congenital progressive hearing impairment, diabetes mellitus, and optic atrophy. The hearing impairment, which is usually diagnosed in the first decade of life, is relatively constant and alters mainly low- and middle-frequency ranges.

Database links:

Entrez Gene: 7466 Human

Entrez Gene: 22393 Mouse

Entrez Gene: 83725 Rat

Omim: 606201 Human

SwissProt: O76024 Human

SwissProt: P56695 Mouse

Unigene: 518602 Human

Unigene: 20916 Mouse

Unigene: 229139 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
版權所有 2004-2026 www.a6308.cn 北京博奧森生物技術有限公司
通過國際質量管理體系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         京公網安備110107000727號
国产精品电影久久 | 欧美日韩一区在线观看 | 99精品无人区乱码在线观看 | 精品国产午夜肉伦伦影院 | 一本大道香蕉久在线播放29 | 一区二区三区日韩免费播放 | 日韩欧美一区二区三区久久婷婷 | 午夜亚洲WWW湿好爽 成人性大片免费观看网站YY | 久久99久久99精品免视看国产四区 | 亚洲国产AV一区二区三区四区 | 久久无码精品一区二区三区 | 免费精品一区二区三区在线观看 | 9191精品国产日本欧美 | 国产无遮挡又黄又爽在线视频 | 完全着衣の爆乳お姉さんが | 狠狠躁日日躁夜夜躁2022麻豆 | 巜豪妇荡乳2在线观看 | 男男H黄动漫啪啪无遮挡网站 | 公交车掀开奶罩边躁狠狠躁视频 | 日韩精品福利视频一区二区三区 | 99狠狠噜夜夜澡人人爽人人爽 | 国产h视频在线观看免费 | 日韩无码高清福利在线看 | 国产呦精品一区二区三区下载 | 韩国美女丝袜一区二区 | 亚洲精品成A人在线观看 | 中文字幕高清在线免费播放 | 最新亚洲中文字幕一区在线 | 国产AV一区二区三区最新精品 | 少妇被黑人到高潮喷出白浆 | 榴莲视频在线看一日韩 | 高清无码视频在线观看 | 国产乱妇乱子视频在线播放国产 | 国产又粗又猛又爽的视频国产 | 四虎永久在线精品国产免费 | 国产日韩精品中文字无码国产精品 | 国产午夜影视大全免费观看 | 一级一级女人18毛片 | 丝袜情趣在线资源二区 | 青青草原综合久久大伊人精品 | 91精品国产制服啪啪无码 |