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- bs-14476R-AP
- 2025年09月30日
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100ul
| 产品编号 | bs-14476R-AP |
| 英文名称 | Dysferlin interacting protein 1 Rabbit pAb, AP conjugated |
| 中文名称 | AP标记的Dysfip1蛋白抗体 |
| 英文别名 | dysferlin interacting protein 1(toonin); Dysfip1; PPR27_HUMAN; Toonin. |
| 产品应用 | WB=1:500-2000 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human Dysferlin interacting protein 1 |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cell Biology > Other Antibodies > Other Antibodies |
| 亚基 | Interacts with DYSF and PPP1CA. |
| 相似性 | Contains 2 ANK repeats. |
| 功能 | Inhibits phosphatase activity of protein phosphatase 1 (PP1) complexes. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 背景资料 | 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). |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |
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Dysferlin interacting protein 1 Rabbit pAb, AP conjugated(bs-14476R-AP)-100ul
￥2980
