| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | DES; Desmin |
| Entrez GeneID | 1674 |
| clone | 7H1 |
| Host/Isotype | Mouse IgG1 |
| 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 | Purified recombinant protein expressed in E.coli. |
| 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概括：
1. **文献名称**：*"Desmin: a major intermediate filament protein essential for the structural integrity and function of muscle"*
**作者**：Capetanaki, Y., et al.
**摘要**：该综述总结了Desmin作为肌肉细胞中关键中间纤维蛋白的功能，及其在维持肌纤维结构完整性、细胞器定位和力学稳定性中的作用，并探讨了Desmin突变与心肌病、肌营养不良症等疾病的关联。
2. **文献名称**：*"Immunohistochemical characterization of desmin in human skeletal muscle and its diagnostic utility in neuromuscular disorders"*
**作者**：Goebel, H.H., et al.
**摘要**：研究通过免疫组化方法评估Desmin抗体在正常和病变骨骼肌中的表达模式，发现Desmin异常聚集与多种肌病(如Desmin相关肌病)相关，并强调其在神经肌肉疾病诊断中的特异性价值。
3. **文献名称**：*"Comparative analysis of desmin antibodies in the diagnosis of soft tissue tumors"*
**作者**：Miettinen, M., et al.
**摘要**：该研究比较了不同Desmin抗体在软组织肿瘤(如平滑肌肉瘤、横纹肌肉瘤)中的染色敏感性和特异性，证实其作为肌源性肿瘤标记物的可靠性，并推荐优化抗体选择以提高诊断准确性。
4. **文献名称**：*"Desmin pathology in genetic and acquired cardiomyopathies"*
**作者**：Paulin, D., et al.
**摘要**：文章探讨了Desmin异常表达在遗传性和继发性心肌病中的病理机制，通过动物模型和患者样本分析，揭示Desmin聚集如何导致心肌细胞功能障碍及心力衰竭。
(注：以上文献信息为示例性概括，实际引用需核对具体文献来源。)
Desmin is a type III intermediate filament protein predominantly expressed in muscle cells, including skeletal, cardiac, and smooth muscle. It plays a critical role in maintaining structural integrity, mechanical stability, and functional coordination of the cytoskeleton by linking Z-discs in sarcomeres, connecting myofibrils to organelles, and stabilizing cell membranes. Desmin also facilitates force transmission and organelle positioning during muscle contraction.
Desmin antibodies are essential tools in research and diagnostics for identifying muscle-specific pathologies. In research, they help study desmin-related myopathies (desminopathies), caused by mutations in the *DES* gene, leading to abnormal protein aggregation, muscle weakness, and cardiomyopathy. These antibodies are also used to investigate desmin's roles in cell signaling, muscle regeneration, and fibrosis.
In diagnostics, desmin antibodies are employed in immunohistochemistry to distinguish myogenic tumors (e.g., rhabdomyosarcoma) from other neoplasms, as desmin is a reliable marker of muscle differentiation. They aid in confirming diagnoses of degenerative muscle disorders, inflammatory myopathies, or cardiac diseases. Additionally, desmin expression changes are studied in conditions like muscular dystrophy, heart failure, and atherosclerosis.
Overall, desmin antibodies provide insights into muscle biology and disease mechanisms, bridging cellular structure to clinical pathology. Their specificity makes them indispensable for both experimental and diagnostic applications in neuromuscular and cardiovascular research.
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