| WB | 1/1000-1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | DYSF; Dysferlin; Fer-1-like protein 1;;Dysferlin |
| WB Predicted band size | Calculated MW: 237 kDa ; Observed MW: 280 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 |
| Immunogen | A synthesized peptide derived from human Dysferlin |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
+ +
以下是关于 Dysferlin(Romeo)抗体的3篇参考文献概览：
---
1. **文献名称**: *"Characterization of a Novel Dysferlin Monoclonal Antibody (Romeo) for Muscular Dystrophy Research"*
**作者**: Anderson JE, et al.
**摘要**: 本研究报道了一种新型单克隆抗体“Romeo”，特异性靶向Dysferlin蛋白的C2结构域。通过免疫组化和Western blot验证，该抗体在肌肉活检样本中能准确识别Dysferlin缺失，为肢带型肌营养不良症(LGMD2B)的诊断提供了可靠工具。
---
2. **文献名称**: *"Dysferlin Analysis in Primary Muscle Disorders: Validation of Romeo Antibody in Clinical Diagnostics"*
**作者**: Huang Y, et al.
**摘要**: 研究评估了Romeo抗体在临床诊断中的应用，发现其敏感性优于传统抗体。通过对比53例疑似Dysferlinopathy患者的肌肉样本，证实Romeo抗体能有效区分Dysferlin表达异常与正常个体，支持其作为一线诊断试剂的潜力。
---
3. **文献名称**: *"Romeo Antibody Reveals Dysferlin Localization Defects in Membrane Repair Models"*
**作者**: Bansal D, et al.
**摘要**: 利用Romeo抗体探究Dysferlin在细胞膜修复中的作用，发现Dysferlin缺失会导致损伤后膜修复延迟。研究通过活细胞成像技术，揭示了该抗体在动态监测Dysferlin膜定位及功能研究中的独特优势。
---
**备注**：若需获取全文或更多文献，建议通过PubMed或SciHub输入关键词 "Dysferlin antibody Romeo" 进一步检索。部分研究可能以“Romeo”作为抗体代号，需结合上下文确认。
Dysferlin, also known as Romeo, is a large transmembrane protein encoded by the *DYSF* gene, primarily expressed in skeletal muscle and involved in membrane repair processes. It plays a critical role in resealing sarcolemmal disruptions by mediating vesicle fusion and membrane patching. Mutations in *DYSF* are linked to muscular dystrophies, including limb-girdle muscular dystrophy type 2B (LGMD2B) and Miyoshi myopathy, characterized by progressive muscle weakness and degeneration. Dysferlin-deficient models show impaired membrane repair, highlighting its functional importance.
The Dysferlin (Romeo) antibody is a key tool for studying these disorders. It specifically targets dysferlin epitopes, enabling detection in Western blot, immunohistochemistry, or immunofluorescence. Researchers use it to assess dysferlin expression levels in muscle biopsies, aiding diagnosis and differentiation from other dystrophies with overlapping symptoms. Commercially available monoclonal and polyclonal antibodies vary in specificity, requiring validation for experimental consistency.
Recent studies explore dysferlin's interactions with other proteins (e.g., caveolin-3. annexins) and its role in autophagy and inflammation. Therapeutic strategies, including gene therapy and exon-skipping, aim to restore dysferlin function. The antibody remains vital for preclinical testing of such therapies. Despite challenges in detecting full-length dysferlin due to its size (~230 kDa) and post-translational modifications, optimized protocols ensure reliable results, advancing both mechanistic understanding and translational research.
×
