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产品简介
| 英文名称 | Anti-Desmin Polyclonal Antibody |
| 别名 | CSM1;CSM2;LGMD2R;DES;Desmin |
| 宿主 | Rabbit |
| 稀释比例 | WB 1:3000. |
| Gene ID | 1674 |
| 保存 | Store at -20°C. Avoid freeze / thaw cycles. |
| Swiss Prot | P17661 |
| 蛋白分子量 | 65kDa |
| 储存液 | Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3. |
| 亚细胞定位 | Cell membrane Cytoplasm sarcolemma |
| 免疫原 | A synthetic peptide of human Desmin |
| 亚型 | IgG |
| 性状 | 液体 |
| 纯化方法 | Affinity purification |
| 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. |
| Public Immunogen Range | 110-160/470aa |
| 克隆类型 | Polyclonal Antibody |
| 交叉反应 | HumanRatMouse |
| 应用 | WB |
| 规格 | 50ul100ul |
注意:
1.本产品仅供科研使用。请勿用于医药、临床诊断或治疗,食品及化妆品等用途。请勿存放于普通住宅区。
2.为了您的安全和健康,请穿好实验服并佩戴一次性手套和口罩操作。
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