| 產(chǎn)品編號 | bs-14476R |
| 英文名稱 | Dysferlin interacting protein 1 Rabbit pAb |
| 中文名稱 | Dysfip1蛋白抗體 |
| 別 名 | dysferlin interacting protein 1(toonin); Dysfip1; PPR27_HUMAN; Toonin. |
| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 克 隆 號 | |
| 交叉反應(yīng) | Mouse,Rat (predicted: Human,Sheep,Cow,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 17 kDa |
| 檢測分子量 | |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Dysferlin interacting protein 1: 7-100/154 |
| 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 |
Dysferlin is a muscle-specific protein that is essential for normal muscle function and development (1). Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.3-p13.1, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM) (1,2). Dysferlin localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle (1,3). Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation (3). Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle (1). It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood (3). Identical mutations in the dysferlin gene can produce more than one myopathy phenotype, indicating that additional genes and/or other factors are also involved in the clinical phenotype (4,5). The DYSF gene has no homology to any other known mammalian gene, but the protein product is related to the spermatogenesis factor fer-1 of Caenorhabditis elegans. The name ‘dysferlin’ combines the role of the gene in producing muscular dystrophy with its homology to C. elegans (6). Function: Inhibits phosphatase activity of protein phosphatase 1 (PP1) complexes. Subunit: Interacts with DYSF and PPP1CA. Similarity: Contains 2 ANK repeats. SWISS: Q86WC6 Gene ID: 116729 Database links: Entrez Gene: 116729 Human Entrez Gene: 68701 Mouse SwissProt: Q86WC6 Human SwissProt: Q9D119 Mouse |
| 產(chǎn)品圖片 |
Sample:
Lane 1: Mouse Large intestine tissue lysates
Lane 2: Rat Large intestine tissue lysates
Primary: Anti-Dysferlin interacting protein 1 (bs-14476R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 17 kDa
Observed band size: 27 kDa
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