FITC标记的扭转蛋白B抗体
产品名称: FITC标记的扭转蛋白B抗体
英文名称: Anti-Torsin B/FITC
产品编号: HZ-
产品价格: null
产品产地: 中国/上海
品牌商标: HZbscience
更新时间: 2023-08-17T10:24:20
使用范围: ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Torsin B/FITC Conjugated antibody
FITC标记的扭转蛋白B抗体
产品编号 | bs-11491R-FITC |
英文名称 | Anti-Torsin B/FITC |
中文名称 | FITC标记的扭转蛋白B抗体 |
别 名 | DQ1; Tor1b; Torsin 1B; TOR1B; FKSG18; Torsin family 1 member B; TOR1B_HUMAN. |
规格价格 | 100ul/2980元 购买 大包装/询价 |
说 明 书 | 100ul |
研究领域 | 细胞生物 神经生物学 |
抗体来源 | Rabbit |
克隆类型 | Polyclonal |
交叉反应 | Human, Mouse, Rat, Dog, Cow, Horse, Sheep, |
产品应用 | 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 | 36kDa |
性 状 | Lyophilized or Liquid |
浓 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Torsin B |
亚 型 | IgG |
纯化方法 | affinity purified by Protein A |
储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
保存条件 | 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. |
产品介绍 | background: TorsinB is a 336 amino acid protein that localizes to the lumen of endoplasmic reticulum and belongs to the Torsin family. Expressed in a variety of tissues with particularly high levels in adult brain, TorsinB is thought to function as a molecular chaperone that assists in the conformational folding of membrane and secreted proteins. Via its ability to regulate protein folding, TorsinB plays a key role in postnatal developmental events and is essential for proper neurological development. TorsinB contains two PKC (protein kinase C) phosphorylations sites, one ATP binding domain and several conserved cysteines and shares 69% amino acid identity with TorsinA, a related family member. Defects in the gene encoding TorsinB are associated with torsion dystonia, an autosomal dominant neurological disorder that is characterized by debilitating muscle contractions throughout the body. Function: Torsins are members of the AAA(+) superfamily of ATPases and are present in all multicellular organisms. Torsin B is an ER protein involved implicated in neurogenesis, neuronal migration, axon/dendrite development and synaptogenesis. Subcellular Location: Endoplasmic reticulum and nuclear envelope Tissue Specificity: Widely expressed with low levels in brain. Not detected in fetal brain. Similarity: Belongs to the clpA/clpB family. Torsin subfamily. Database links: Entrez Gene: 27348 Human Entrez Gene: 30934 Mouse Omim: 608050 Human SwissProt: O14657 Human SwissProt: Q9ER41 Mouse Unigene: 252682 Human Unigene: 249164 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
TrSiNB是一种定位于内质网内腔的336氨基酸蛋白,属于Torsin家族。TrSiNB在脑中具有特别高的水平的各种组织中表达,被认为起到协助膜和分泌蛋白构象折叠的分子伴侣的作用。通过调节蛋白质折叠的能力,TorsinB在出生后的发育事件中起着关键的作用,并且对于正确的神经发育是必不可少的。TrONSB包含两个PKC(蛋白激酶C)磷酸化位点,一个ATP结合域和几个保守半胱氨酸,与69%个氨基酸同一个家族成员TorsinA共享。TursNB基因的缺陷与扭转性肌张力障碍有关,它是一种常染色体显性神经系统疾病,其特征是全身衰弱的肌肉收缩。