97久久久精品综合88久久_亚洲国产精品一_久热热国产久热_97操操操_北条麻妃在线免费观看_精品国自产拍天天拍

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  聯系我們
а的天堂网最新版在线,少妇放荡的呻吟干柴烈火动漫,噜啦噜色姑娘综合
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-WFS1/AP Conjugated antibody (bs-11272R-AP)
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-AP
英文名稱 Rabbit Anti-WFS1/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)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 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=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.kastlife.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
主站蜘蛛池模板: 欧美成亚洲 | 学生在线精品一区二区 | 91丨porny在线牛牛影视 | 中文字幕在线一区二区三区 | 久久久久久国产精品免费免费狐狸 | 成年人免费观看网站 | 国产成人AV性色在线影院色戒 | 在线免费看av的网站 | 中文字幕在线中文字幕二区 | 毛片免费毛片一级jjj毛片 | 视频免费观看的网站 | 国产福利男女XX00视频 | 亚洲区激情区无码区日韩区 | 爽好久久久欧美精品 | 亚洲AV成人一区二区三区天堂 | 波多野结衣在线视频一区二区三区 | 国产v精品成人免费视频400条 | 禁断一区二区三区在线 | 99国产观看免费视频 | 视频一区视频二区视频 | 国产精品成人免费精品自在线观看 | 丰满少妇被猛烈进出69影院 | 蜜乳av另类精品一区二区 | 无码AV无码一区二区桃花岛 | xxx麻豆| 91精品国产综合久久久久久首页 | 99免费精品视频 | 亚洲日韩色在线影院性色 | 国产精品免费网站 | 亚洲色偷偷av男人的天堂 | 日本网站在线观看一区二区三区 | 亚洲国产日韩a综合在线 | 一区二区在线播放视频 | 亚洲精品无码久久久久去Q 国产精选一级毛片 | 波多野结衣永久免费视频 | 亚洲日韩在线中文字幕线路2区 | www.成人在线 | 风韵丰满熟妇啪啪区老老熟女百度 | 日韩视频一二三 | 亚洲一区天堂 | 少妇把腿扒开让我添69 |