| 產(chǎn)品編號(hào) | bs-2401R-BF488 |
| 英文名稱1 | Rabbit Anti-Nebulin/BF488 Conjugated antibody |
| 中文名稱 | BF488標(biāo)記的伴肌動(dòng)蛋白抗體 |
| 別 名 | DKFZp686C1456; FLJ11505; FLJ36536; FLJ39568; FLJ39584; NEB 177D; NEB; NEB177D; NEM 2; NEM2; NEBU_HUMAN; FLJ11505. |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買 大包裝/詢價(jià) |
| 說(shuō) 明 書 | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 結(jié)合蛋白 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, ) |
| 產(chǎn)品應(yīng)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 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Nebulin |
| 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: 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 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. 具有穩(wěn)定細(xì)肌絲的作用。 |
