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Anti-Dysferlin interacting protein 1 Polyclonal Antibody 是一种 Rabbit 抗体,靶向 Dysferlin interacting protein 1。Anti-Dysferlin interacting protein 1 Polyclonal Antibody 可用于 WB。
Anti-Dysferlin interacting protein 1 Polyclonal Antibody 是一种 Rabbit 抗体,靶向 Dysferlin interacting protein 1。Anti-Dysferlin interacting protein 1 Polyclonal Antibody 可用于 WB。
| 规格 | 价格 | 库存 | 数量 |
|---|---|---|---|
| 50 μL | ¥ 1,170 | 5日内发货 | |
| 100 μL | ¥ 1,970 | 5日内发货 | |
| 200 μL | ¥ 2,795 | 5日内发货 |
| 产品描述 | Anti-Dysferlin interacting protein 1 Polyclonal Antibody is a Rabbit antibody targeting Dysferlin interacting protein 1. Anti-Dysferlin interacting protein 1 Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| 反应种属 | Mouse,Rat (predicted:Human,Dog,Cow,Horse,Sheep) |
| 应用 | WB |
| 推荐剂量 | WB: 1:500-2000 |
| 抗体种类 | Polyclonal |
| 宿主来源 | Rabbit |
| 构建方式 | Hybridoma Polyclonal Antibody |
| 纯化方式 | Protein A purified |
| 性状 | Liquid |
| 缓冲液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 浓度 | 1mg/ml |
| 研究背景 | 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). |
| 免疫原 | KLH conjugated synthetic peptide: human Dysferlin interacting protein 1 |
| 抗原种属 | Human |
| 基因名称 | PPP1R27 |
| 基因ID | |
| 蛋白名称 | Protein phosphatase 1 regulatory subunit 27 |
| Uniprot ID | |
| 功能 | Inhibits phosphatase activity of protein phosphatase 1 (PP1) complexes. |
| 分子量 | Theoretical: 17 kDa. |
| 储存方式 | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| 运输方式 | Shipping with blue ice. |
| 存储 | store at low temperature | -20°C for 1 year |