Desmin (ABT168) Mouse mAb (Ready to Use)
- 货号:YM6974R
- 应用:IHC
- 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:
- CMD1I;CSM1;CSM2;DES;DESM_HUMAN;Desmin;FLJ12025;FLJ39719;FLJ41013;FLJ41793;Intermediate filament protein;OTTHUMP00000064865
- Human Swiss Prot No:
- P17661
- Mouse Swiss Prot No:
- P31001
- Rat Swiss Prot No:
- P48675
- 免疫原:
- Synthesized peptide derived from human Desmin AA range: 400-470
- 特异性:
- The antibody can specifically recognize human Desmin protein.
- 组成:
- PBS, pH7.2, 0.03% Porcolin 300, containing stabilizing protein
- 来源:
- Monoclonal Mouse IgG2b, Kappa
- 稀释:
- Ready to use for IHC
- 纯化工艺:
- The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
- 储存:
- 2°C to 8°C/1 year
- 其他名称:
- CMD1I;CSM1;CSM2;DES;DESM_HUMAN;Desmin;FLJ12025;FLJ39719;FLJ41013;FLJ41793;Intermediate filament protein;OTTHUMP00000064865
- 分子量:
- 52kD
- 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:
- Appendix/ Colon
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- 产品图片
- Human endometrial adenocarcinoma tissue was stained with Anti-Desmin (ABT168) Antibody
- Human prostatic adenocarcinoma tissue was stained with Anti-Desmin (ABT168) Antibody
- Human smooth muscle tissue was stained with Anti-Desmin (ABT168) Antibody
- Human stomach tissue was stained with Anti-Desmin (ABT168) Antibody
- Human tonsil tissue was stained with Anti-Desmin (ABT168) Antibody