FITC标记的肌营养不良蛋白相关蛋白1抗体
产品名称： FITC标记的肌营养不良蛋白相关蛋白1抗体
英文名称： Anti-Utrophin/FITC
产品编号： HZ-20432R-FITC
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-Utrophin/FITC Conjugated antibody
FITC标记的肌营养不良蛋白相关蛋白1抗体
| 英文名称 | Anti-Utrophin/FITC |
| 中文名称 | FITC标记的肌营养不良蛋白相关蛋白1抗体 |
| 别 名 | DMDL; DRP 1; DRP; DRP-1; DRP1; Dystrophin like protein; Dystrophin related protein 1; Dystrophin related protein; Dystrophin-related protein 1; FLJ23678; UTRN; UTRO_HUMAN; Utrophin (homologous to dystrophin); Utrophin |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, 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 | 394kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 2mg/1ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Utrophin |
| 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: Dystrophin and utrophin are related structural, Actin-binding proteins that are involved in anchoring the cytoskeleton to the plasma membrane. Dystrophin is the protein product of the Duchenne/Becker muscular dystrophy gene. Dystrophin expression is found in muscle and brain tissues, where it is localized to the inner surface of the plasma membrane. It has been speculated that alternative splicing of the carboxy terminus allows dystrophin to interact with a variety of proteins. Research has shown that the loss of dystrophin-associated proteins in Duchenne afflicted muscle is due to the absence of dystrophin rather than to muscle degradation and that the lack of dystrophin results in the loss of linkage between the cytoskeleton and the extracellular matrix. Evidence suggests that the upregulation of utrophin can reduce the dystrophic pathology. Function: May play a role in anchoring the cytoskeleton to the plasma membrane. Subunit: Interacts with the syntrophins SNTA1; SNTB1 and SNTB2. Interacts with SYNM. Interacts (via its WWW and ZZ domains) with DAG1 (via the PPXY motif of betaDAG1); the interaction is inhibited by the tyrosine phosphorylation of the PPXY motif of DAG1. Subcellular Location: Cell junction > synapse > postsynaptic cell membrane. Cytoplasm > cytoskeleton. Neuromuscular junction. Tissue Specificity: Muscle. Similarity: Contains 2 CH (calponin-homology) domains. Contains 20 spectrin repeats. Contains 1 WW domain. Contains 1 ZZ-type zinc finger. Database links: HGNC: 12635human
Entrez Gene: 7402human Ensembl: ENSG00000152818human OMIM: 128240human UniProtKB: P46939human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
肌营养不良素和肌动蛋白是相关的结构，肌动蛋白结合蛋白，参与锚定细胞骨架到质膜。肌营养不良蛋白是Duchenne／贝克尔肌营养不良基因的蛋白产物。肌营养不良蛋白在肌肉和脑组织中表达，定位于质膜的内表面。据推测，羧基末端的替代性剪接允许肌营养不良蛋白与各种蛋白质相互作用。研究表明，在Duchenne受损的肌肉中肌营养不良蛋白相关蛋白的丢失是由于缺乏肌营养不良蛋白而不是由于肌肉降解，并且缺乏肌营养不良蛋白导致细胞骨架和细胞外基质之间的连接丢失。有证据表明，上调UTROLIN可以减少营养不良的病理。