Desmin (ABT19R) rabbit mAb
- 货号:YM7110
- 应用:IHC;WB; ELISA
- 种属:Human;Mouse;(predicted: Rat)
- 靶点:
- Desmin
- 简介:
- >>Hypertrophic cardiomyopathy;>>Arrhythmogenic right ventricular cardiomyopathy;>>Dilated cardiomyopathy
- 基因名称:
- DES
- 蛋白名称:
- Desmin
- Human Gene Id:
- 1674
- Human Swiss Prot No:
- P17661
- 免疫原:
- Synthesized peptide derived from human Desmin AA range:400-470
- 特异性:
- This antibody detects endogenous levels of Desmin
- 组成:
- PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
- 来源:
- Monoclonal, Rabbit IgG1, Kappa
- 稀释:
- IHC 1:100-500, WB 1:500-1000, ELISA 1:5000-20000
- 纯化工艺:
- Recombinant Expression and Affinity purified
- 储存:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称:
- CMD1I;CSM1;CSM2;DES;DESM_HUMAN;Desmin;FLJ12025;FLJ39719;FLJ41013;FLJ41793;Intermediate filament protein;OTTHUMP00000064865
- 分子量:
- 54kD
- 背景:
- This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008],
- 功能:
- disease:Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. 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.,disease:Defects in DES are the cause of desmin-related cardio-skeletal myopathy (CSM) [MIM:601419]; 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).,disease:Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400].
- 细胞定位:
- Cytoplasmic
- 组织表达:
- Cytoplasmic
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