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(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
女人18毛片A级毛片嫰阝,特a级黄色片,欧美精品1区2区3区
Rabbit Anti-WFS1/Cy3 Conjugated antibody (bs-11272R-Cy3)
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(jià)
產(chǎn)品編號 bs-11272R-Cy3
英文名稱 Rabbit Anti-WFS1/Cy3 Conjugated antibody
中文名稱 Cy3標(biāo)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(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  神經(jīng)生物學(xué)  信號轉(zhuǎn)導(dǎo)  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(yīng) (predicted: Human, Mouse, Rat, Dog, Cow, Horse, )
產(chǎn)品應(yīng)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(chǔ) 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:
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(quán)所有 2004-2026 www.ggggww.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
主站蜘蛛池模板: 亚洲日韩欧美一区二区三区 | 18禁美女裸体网站无遮挡 | 亚洲欧美日韩精品在线 | 日韩精品一区二区亚洲AV | 日韩av在线网 | 99精品在线 | 日韩精品人妻系列无码专区免费 | 亚洲最大的av网站 | 蜜臀av性久久久久蜜臀aⅴ流畅 | 嗯快点别停舒服好爽受不了了 | 48久久国产精品性色aⅴ人妻 | 不卡一不卡二不卡三 | 国产日本亚洲香蕉视频 | 丁香婷婷深爱五月亚洲综合 | 精品欧洲AV无码一区二区三区 | 特级做a爰片毛片免费看 | 成人一级片在线免费观看 | 乱人伦中文无码视频在线观看 | 国产成人无码精品久久久小说 | 国产成人亚洲精品影院 | 一级免费黄色毛片 | 国产精品白丝av在线观看播放 | 高清毛片在线观看 | 黄色国产免费看 | 国产精品一品二区三区四区18 | 午夜伦yy44880影院 | 欧美中文字幕 | 狠狠干导航 | 国产精品视频二区三区 | 波多野结衣AV一区二区无码 | 亚洲欧洲日产国码无码久久99 | 国产美女在线精品 | 在线观看日本高清二区 | 国产综合视频一区二区三区免费 | av在线影院| 国产成人精| 日韩在线免费播放 | 亚洲AV成人综合网久久 | 国产尤物在线视频 | 嗯快点别停舒服好爽受不了了 | 国产999精品久久久久久 |