Dysfip1蛋白抗体
规格：1mg/1ml
英文名： Dysferlin interacting protein 1
别名： dysferlin interacting protein 1 (toonin); Dysfip1; PPR27_HUMAN; Toonin.
分子量： 17kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Dysferli
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Sheep,
细胞定位：
Dysfip1蛋白抗体产品介绍：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, *****-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 Dysfip1蛋白抗体(1). It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in *****hood (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 isDysfip1蛋白抗体 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. Gene ID: 116729 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.
Dysfip1蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid