FITC标记的伴肌动蛋白抗体
产品名称： FITC标记的伴肌动蛋白抗体
英文名称： Anti-Nebulin/FITC
产品编号： HZ-2401R-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： IF=1:50-200
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Rabbit Anti-Nebulin/FITC Conjugated antibody
FITC标记的伴肌动蛋白抗体
| 英文名称 | Anti-Nebulin/FITC |
| 中文名称 | FITC标记的伴肌动蛋白抗体 |
| 别 名 | DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505. |
| 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 免疫学 结合蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 500-800kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Nebulin |
| 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: Nebulin is a high molecular weight protein (500-800 kD), localized at the N2-line of skeletal muscle myofibrils. It exhibits an elastic stretch dependence, as it maintains the same proportional distance between the z-line and the m-line as the sarcomere is stretched. Function: This giant muscle protein may be involved in maintaining the structural integrity of sarcomeres and the membrane system associated with the myofibrils. Bind and stabilize F-actin. Subcellular Location: Cytoplasm > myofibril > sarcomere. Cytoplasm > cytoskeleton. Tissue Specificity: Muscle specific. Located in the thin filament of striated muscle. DISEASE: Defects in NEB are the cause of nemaline myopathy type 2 (NEM2) [MIM:256030]. A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. Similarity: Contains 178 nebulin repeats. Contains 1 SH3 domain. Database links: Entrez Gene: 4703 Human Entrez Gene: 17996 Mouse Entrez Gene: 311029 Rat Omim: 161650 Human SwissProt: P20929 Human Unigene: 588655 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 具有稳定细肌丝的作用 |
星云蛋白是一种高分子量蛋白质（500～800 kD），定位于骨骼肌肌原纤维的N2系。它表现出弹性拉伸依赖性，因为它保持在Z线和M线之间的相同的比例距离，因为肌节伸展。