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PPP1R27 Antibody Blocking Pept

ide(bs-14476P)-500ug
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  • 2025年10月16日
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      500ug

    产品编号bs-14476P
    英文名称PPP1R27 Antibody Blocking Peptide
    中文名称Dysfip1蛋白封闭多肽
    英文别名dysferlin interacting protein 1 (toonin); Dysfip1; PPR27_HUMAN; Toonin.
    纯化方法HPLC
    研究领域

    Cell Biology > Other Antibodies > Other Antibodies

    亚基Interacts with DYSF and PPP1CA.
    相似性Contains 2 ANK repeats.
    功能Inhibits phosphatase activity of protein phosphatase 1 (PP1) complexes.
    保存条件Shipped at 4℃. Stored 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.
    背景资料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).

     

     

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