Anti-Dysferlin interacting protein 1/FITC Conjugated抗体
产品名称： Anti-Dysferlin interacting protein 1/FITC Conjugated抗体
英文名称： Anti-Dysferlin interacting protein 1/FITC
产品编号： YB--14476R-FITC
产品价格： null
产品产地： 中国/美国
品牌商标： Ybscience
更新时间： 2023-08-17T10:29:50
使用范围： 科研使用
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Anti-Dysferlin interacting protein 1/FITC Conjugated抗体
| 产品编号 | YB-14476R-FITC |
| 英文名称 | Anti-Dysferlin interacting protein 1/FITC |
| 中文名称 | FITC标记的Dysfip1蛋白抗体 |
| 别 名 | dysferlin interacting protein 1 (toonin); Dysfip1; PPR27_HUMAN; Toonin. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 免疫学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Cow, Horse, Sheep, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 17kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 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 |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: 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. Database links: Entrez Gene: 116729 Human Entrez Gene: 616223 Cow Entrez Gene: 608003 Dog Entrez Gene: 68701 Mouse Entrez Gene: 287881 Rat SwissProt: Q86WC6 Human SwissProt: Q9D119 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |