| 产品编号 | bs-14476R |
| 英文名称 | Dysferlin interacting protein 1 Rabbit pAb |
| 中文名称 | Dysfip1蛋白抗体 |
| 别 名 | dysferlin interacting protein 1(toonin); Dysfip1; PPR27_HUMAN; Toonin. |
| 研究领域 | 细胞生物 免疫学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 克 隆 号 | |
| 交叉反应 | Mouse,Rat (predicted: Human,Sheep,Cow,Dog,Horse) |
| 产品应用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 17 kDa |
| 检测分子量 | |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Dysferlin interacting protein 1: 7-100/154 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
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 |
| 产品图片 |
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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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
