| 产品编号 | bs-11272R |
| 英文名称 | WFS1 Rabbit pAb |
| 中文名称 | Wolfram综合征蛋白1抗体 |
| 别 名 | DFNA14; DFNA38; DFNA6; DIDMOAD; WFRS; WFS; Wolfram syndrome 1(wolframin); Wolfram syndrome; WOLFRAMIN; WFS1_HUMAN. |
| 研究领域 | 肿瘤 神经生物学 信号转导 生长因子和激素 糖尿病 新陈代谢 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human (predicted: Mouse,Rat,Cow,Dog,Horse) |
| 产品应用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 97 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞浆 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human WFS1: 791-890/890 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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. SWISS: O76024 Gene ID: 7466 Database links: Entrez Gene: 7466 Human Entrez Gene: 22393 Mouse Omim: 606201 Human SwissProt: O76024 Human SwissProt: P56695 Mouse Unigene: 518602 Human Unigene: 20916 Mouse Unigene: 229139 Rat |
| 产品图片 |
Sample:
HepG2 Cell （Human）Lysate at 40 ug
Raji Cell （Human）Lysate at 40 ug
Primary: Anti-WFS1 (bs-11272R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 97 kD
Observed band size: 105 kD
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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
