-
Dysferlin Antibody
Dysferlin Antibody
- 中文名称：
- Dysferlin Antibody
- 英文名称：
- Dysferlin Antibody
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA30280
- 规格：
- 0.05 mL|0.1 mL|2x0.1 mL|3x0.1 mL|4x0.1 mL
- 保存建议：
- 储存在-20摄氏度
- 货期：
- 6-8周
- 来源宿主：
- Rabbit
- 反应种属：
- Human, Mouse
- 应用：
- WB (Western Blot), ICC (Immunocytochemistry), IF (Immunofluorescence), IHC (Immunohistochemistry)
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Monoclonal
同型:N/A
克隆号:[JF05-54]
特异性:N/A
纯度:ProA affinity purified
形式：1*TBS (pH7.4), 1% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
浓度：N/A
- 说明书：
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.
