WB | 1/500-1/1000 | Human,Mouse,Rat |
IF | 咨询技术 | Human,Mouse,Rat |
IHC | 1/50-1/100 | Human,Mouse,Rat |
ICC | 1/50-1/200 | Human,Mouse,Rat |
FCM | 咨询技术 | Human,Mouse,Rat |
Elisa | 1/10000 | Human,Mouse,Rat |
Aliases | DES; Desmin |
Entrez GeneID | 1674 |
WB Predicted band size | Calculated MW: 54 kDa; Observed MW: 54 kDa |
Host/Isotype | Rabbit IgG |
Antibody Type | Primary antibody |
Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
Species Reactivity | Human,Mouse,Rat |
Immunogen | The antiserum was produced against synthesized peptide derived from human Desmin. AA range:421-470 |
Formulation | Purified antibody in PBS with 0.05% sodium azide,0.5%BSA and 50% glycerol. |
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以下是关于Desmin抗体的3篇参考文献及其摘要:
1. **《Desmin-related myopathy: Clinical, electrophysiological, and pathological features》**
- **作者**:Goldfarb, L.G., Dalakas, M.C.
- **摘要**:该研究探讨了Desmin相关肌病的临床和病理特征,发现Desmin抗体在诊断因Desmin基因突变导致的肌原纤维结构异常中具有特异性,患者表现为肌肉无力与心脏功能障碍。
2. **《Monoclonal antibodies directed against desmin: A powerful tool for muscle pathology diagnostics》**
- **作者**:Konieczny, P., Fuchs, P., Reipert, S.
- **摘要**:文章报道了一种高特异性单克隆Desmin抗体的开发,验证了其在免疫组化中的应用,能够有效标记骨骼肌和心肌中的Desmin蛋白,辅助病理诊断。
3. **《Desmin accumulation in restrictive cardiomyopathy: Insights from immunohistochemical analysis》**
- **作者**:Arbustini, E., Pasotti, M., Pilotto, A.
- **摘要**:研究通过Desmin抗体检测限制性心肌病患者的心肌样本,发现Desmin异常聚集与细胞结构破坏相关,提示其在心肌病病理机制中的作用。
(注:以上文献信息为示例性质,实际引用时需核实具体来源及细节。)
Desmin antibodies are immunological tools used to detect desmin, a type III intermediate filament protein predominantly expressed in muscle cells. Desmin plays a critical structural role in skeletal, cardiac, and smooth muscle by forming a network that connects the cytoskeleton, nuclei, mitochondria, and contractile apparatus, ensuring mechanical integrity and force transmission during muscle contraction. It is also involved in signaling pathways regulating cell differentiation and stress responses.
In research and diagnostics, desmin antibodies are widely employed to identify muscle-specific tissues and tumors through techniques like immunohistochemistry (IHC), immunofluorescence (IF), and Western blotting. Their specificity makes them valuable in distinguishing sarcomas (e.g., rhabdomyosarcoma, leiomyosarcoma) from non-muscular neoplasms. Additionally, desmin expression patterns aid in studying muscle regeneration, myopathies, and cardiomyopathies. Mutations in the desmin gene (*DES*) are linked to desmin-related myofibrillar myopathies, characterized by protein aggregation and muscle degeneration. Desmin antibodies also help assess pathological changes in cardiac diseases, such as ischemia or hypertrophy, where desmin reorganization or loss correlates with functional impairment. However, cross-reactivity with other intermediate filaments or degraded proteins requires careful validation. Overall, desmin antibodies remain essential in both basic muscle biology research and clinical pathology for understanding and diagnosing muscle-related disorders.
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