| 產(chǎn)品編號(hào) | bs-2401R |
| 英文名稱 | Rabbit Anti-Nebulin antibody |
| 中文名稱 | 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. |
| 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,Rabbit,Pig,Cow,Chicken,Dog) |
| 產(chǎn)品應(yīng)用 | ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Nebulin: 6401-6669/6669 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. SWISS: P20929 Gene ID: 4703 Database links: Entrez Gene: 4703 Human Entrez Gene: 17996 Mouse Omim: 161650 Human SwissProt: P20929 Human Unigene: 588655 Human 具有穩(wěn)定細(xì)肌絲的作用。 |
