北京索莱宝科技
专注于生物学试剂及试剂盒领域
服务热线：18101056239
专注于生物学试剂及试剂盒领域
服务热线：18101056239
产品型号：K106550P
更新时间：2022-08-15
厂商性质：生产厂家
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Anti-Desmin Polyclonal Antibody
| 英文名称 | Anti-Desmin Polyclonal Antibody |
|---|---|
| 宿主 | Rabbit |
| 别名 | CSM1;CSM2;LGMD2R;DES;Desmin |
| 应用 | WB |
| 稀释比例 | WB 1:3000. |
| 交叉反应 | Human Mouse Rat |
| 蛋白分子量 | 65kDa |
| Gene ID | 1674 |
| 保存 | Store at -20°C. Avoid freeze / thaw cycles. |
| 储存液 | Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3. |
| 纯化方法 | Affinity purification |
| 亚型 | IgG |
| 免疫原 | A synthetic peptide of human Desmin |
| 性状 | 液体 |
| Public Immunogen Range | A synthetic peptide of human Desmin |
| Subcellular Locations | Cell membrane Cytoplasm sarcolemma |
| Swiss Prot | P17661 |
| 克隆类型 | Polyclonal Antibody |
| 背景资料 | Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. |
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