- 中文名称
Dysferlin
- 英文名字
- Dysferlin
- 供应商
- Nordic BioSite
- 产品货号
- NDC-BT-P3VTHW-50
- 产品报价
- ￥询价/50ul
- 产品说明书
- 点击查看
- 购买方式
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- 产品新闻
- 背景资料
- Dysferlin is a muscle-specific protein that is essential for normal muscle function and development. Mutations in the human dysferlin gene, DYSF, which maps to chromosome 2p13.2, are associated with limb girdle muscular dystrophy-2B (LGMD-2B) and a related, adult-onset, distal dystrophy known as Miyoshi myopathy (MM). Dysferlin localizes to the muscle fiber membrane, but is absent in MM and LGMD-2B muscle. Dysferlin is detected in 5-6 week embryos, when limbs begin to form regional differentiation. Although it is not essential for initial myogenesis, dysferlin appears to be critical for sustained normal function in mature muscle. It has been suggested that the absence of dysferlin during development gives rise to the disease phenotype in adulthood. 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. 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.
- 应用类型
- WB,ICC,IF,IHC
- 免疫原
- recombinant protein
- 来源宿主
- rabbit
- 反应性
- human,mouse
- 保存建议
- Store at +4
- 其他
- Nordic BioSite(北欧生物) 总部位于瑞典，成立于1997年，旨在分销研究和诊断领域的高质量和创新产品。 Nordic BioSite 被公认为整个北欧生物医学研究和诊断产品领域的领导者。他们拥有超过570万种的产品组合，代理了遍布欧洲和美国的顶级生物试剂制造商，是欧洲知名的一站式生物试剂代理商。除了作为分销商之外，Nordic BioSite 还拥有不断增长的自产产品线——BioSite mAb、BioSite ELISA、BioSite Flow、Optibodies和 BioSite Protein, 目前艾美捷代理的是Nordic BioSite自产产品线(共26W+产品)。Nordic BioSite的产品类型主要是抗体，蛋白和试剂盒，产品适用范围涵盖15个不同的物种(人，牛，犬，鲶鱼，鸡，海豚，马，猫，豚鼠，小鼠，绵羊，兔子，大鼠，猪和火鸡)，可为您提供一站式的生命科学研究解决方案~
- 注意
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该页面的中文产品信息的翻译，仅供参考。准确的产品信息请以厂家的英文说明书为准。下单前，请浏览说明书确认。
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