- 中文名称
DMD 抗体 - C-末端区域 (ARP79548_P050)
- 英文名字
- DMD Antibody - C-terminal region (ARP79548_P050)
- 供应商
- Aviva Systems Biology
- 产品货号
- aviva-ARP79548-P050
- 产品报价
- ¥询价/100ul

- 产品说明书
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- 购买方式
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- 产品新闻

- 背景资料
- The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix.
- 应用类型
- WB
- 免疫原
- The immunogen is a synthetic peptide directed towards the C terminal region of human DMD
- 来源宿主
- Rabbit
- 反应性
- Human
- 保存建议
- For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
- 其他
- Aviva Systems Biology总部位于加利福尼亚州圣迭戈,在中国北京设有办公室,专注于为研究需求提供多克隆和单克隆抗体、ELISA试剂盒、蛋白质和定制服务。Aviva Systems Biology生产了24,000种经过验证的多克隆抗体,并提供近20,000种ELISA试剂盒,定制实验室服务包括蛋白表达和纯化、抗体开发,以及ELISA的开发、验证和生产。Aviva Systems Biology为与独特物种和靶标相关的研究提供独特工具,研究领域包括转录因子、癌症、心血管、细胞生物学、DNA损伤和修复、表观遗传学、信号转导、细胞分化、干细胞生物学等等。

- 注意
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