北京索萊寶科技
專注于生物學試劑及試劑盒領域
服務熱線：18101056239
專注于生物學試劑及試劑盒領域
服務熱線：18101056239
產品型號：K106550P
更新時間：2022-08-15
廠商性質：生產廠家
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產品分類
Anti-Desmin Polyclonal Antibody
| 英文名稱 | Anti-Desmin Polyclonal Antibody |
|---|---|
| 宿主 | Rabbit |
| 別名 | CSM1;CSM2;LGMD2R;DES;Desmin |
| 應用 | WB |
| 稀釋比例 | WB 1:3000. |
| 交叉反應 | Human Mouse Rat |
| 蛋白分子量 | 65kDa |
| Gene ID | 1674 |
| 保存 | Store at -20°C. Avoid freeze / thaw cycles. |
| 儲存液 | Buffer: PBS with 0.03% Proclin300, 50% glycerol, pH7.3. |
| 純化方法 | Affinity purification |
| 亞型 | IgG |
| 免疫原 | A synthetic peptide of human Desmin |
| 性狀 | 液體 |
| Public Immunogen Range | A synthetic peptide of human Desmin |
| Subcellular Locations | Cell membrane Cytoplasm sarcolemma |
| Swiss Prot | P17661 |
| 克隆類型 | Polyclonal Antibody |
| 背景資料 | 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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