超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

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
91精品国自产在线观看欧美,免费无码百合真人片18禁,五月婷婷天堂
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-WFS1/BF488 Conjugated antibody (bs-11272R-BF488)
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-BF488
英文名稱 Rabbit Anti-WFS1/BF488 Conjugated antibody
中文名稱 BF488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 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.ggggww.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: 国产日韩在线看 | 猫咪永久入口网址 | 啦啦啦www在线观看免费视频 | 天啦噜国产精品亚洲精品 | 国产一区二区三区美女 | 女性裸体无遮挡无遮掩视频蜜芽 | 宝贝张开腿嗯啊高潮了视频 | 欧美A级理论片在线播放 | 精品少妇爆乳无码专区久久 | 日韩v亚洲v欧美v精品综合 | 吴梦梦在线看 | 日韩最新中文字幕 | 日韩欧美国产一区二区三区 | 成人午夜视频免费 | 亚洲一区二区三区在线观看网站 | 成人超碰在线观看 | 国产91精品久久久久久久网曝门 | 好好的日在线视频观看 | 国产精品无码日韩字幕资不卡 | 欧美色视频在线观看 | 久久久久久伊人 | 自拍日韩亚洲一区在线 | 天天爽影院一区二区在线影院 | 国产欧美一区二区三区精品酒店 | 成年人网站国产 | 精品人伦一区二区三区蜜桃免费 | 日日夜精品欧洲日日噜噜 | 网曝门国产一区二区三区四区 | 相泽南av日韩在线 | 丁香婷婷深爱五月亚洲综合 | 国产香蕉97碰碰久久人人九色 | 成年视频女人的天堂天天看片 | 天天操综合视频 | 中文字幕久久精品波多野结百度 | 久草视频手机在线观看 | 91精品国产乱码久久久久久久久 | 日韩五码在线观看 | 国产一区三区四区 | 亚洲品质自拍视频 | 91精品久久久久五月天精品 | 六十路の高齢熟女が |