| 產(chǎn)品編號 | bs-14476R-Gold |
| 英文名稱 | Rabbit Anti-Dysferlin interacting protein 1/Gold Conjugated antibody |
| 中文名稱 | 膠體金標記的Dysfip1蛋白抗體 |
| 別 名 | dysferlin interacting protein 1 (toonin); Dysfip1; PPR27_HUMAN; Toonin. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul（10nm 15nm 35nm） |
| 研究領域 | 細胞生物 免疫學 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應 | (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Sheep, ) |
| 產(chǎn)品應用 | IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 17kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 0.4mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Dysferlin interacting protein 1 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. |
| 保存條件 | Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles. |
| 產(chǎn)品介紹 |
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: 68701 Mouse 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. |
| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關于肽鏈的設計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
