| 克隆类型 ： |
Rabbit Polyclonal Antibody |
|
亚 型 ： |
Rabbit Ig |
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免 疫 原 ： |
KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of human desmin. |
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产品描述 ： |
Desmin is a class-III intermediate filament found in muscle cells. In adult striated muscle desmin forms a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. Defects in desmin are the cause of myopathy myofibrillar desmin-related (MFM-DES), also known as desmin-related myopathy (DRM). DRM is neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. Defects in desmin are the cause of cardiomyopathy dilated type 1I (CMD1I). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. |
| 适用物种 ： |
H, M, R |
|
应用范围 ： |
E, WB, IHC |
| 应用说明 ： |
E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500 |
| 别 名 ： |
DES; Desmin |
|
形 态 ： |
Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4 with 0.09% sodium azide. Antibody was purified by Protein-G affinity chromatography. |
| 保存条件 ： |
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment. |
| 产品链接 ： |
http://www.abbiotec.com/antibodies/desmin-antibody |
| 标 记 物 ： |
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产品说明 ： |
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