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Antibody Product Overview
| ELK.No | ES20865 |
| Product name | Desmin Rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | IHC;WB |
| Other name | Desmin |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 1674 |
| Human Swiss-Prot | P17661 |
| Source | Rabbit |
| Isotype | IgG |
| Target | Desmin |
| Fields | >>Hypertrophic cardiomyopathy;>>Arrhythmogenic right ventricular cardiomyopathy;>>Dilated cardiomyopathy |
| Gene name | DES |
| Protein name | Desmin |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 13346 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P31001 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 64362 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | P48675 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human Desmin AA range: 197-247 |
| Specificity | This antibody detects endogenous levels of Desmin at Human, Mouse,Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.75% sodium azide. |
| Clonality | Polyclonal |
| Dilution | IHC-p1:50-200 ,WB 1:1000-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 54kD |
| Background | 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], |
| Function | 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]. |
| Subcellular location | Cytoplasmic |
| Expression | Muscle,Skeletal muscle, |
Additional Images
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Western blot analysis of 1)Mouse Heart Tissue, 2) Rat Heart Tissue Lysate using DesminRabbit Polyclonal Antibody diluted at 1:2,000.
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Immunohistochemical analysis of paraffin-embedded human liver cancer. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).
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: AO-06-ES20865-50
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