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Rabbit Polyclonal DMD Antibody

  • 中文名: DMD抗体
  • 别    名: BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
货号: IPDX08256
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/25-1/100 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/1000-1/2000 Human,Mouse,Rat

产品详情

AliasesBMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse
ImmunogenFusion protein of human DMD
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是关于DMD(杜氏肌营养不良症)抗体的3篇参考文献及其简要摘要:

1. **"Antibody-based diagnosis of Duchenne muscular dystrophy by dystrophin quantification in blood"**

*作者:Hoffman EP, et al.*

**摘要**:该研究开发了一种基于抗体的免疫检测方法,通过检测血液中抗肌萎缩蛋白(dystrophin)的含量,为非侵入性诊断DMD提供新策略。

2. **"Exon skipping and dystrophin restoration in DMD patient muscle cells following treatment with antisense oligonucleotides"**

*作者:Anthony K, et al.*

**摘要**:研究利用特异性抗体检测外显子跳跃治疗后患者肌细胞中截短型dystrophin蛋白的表达,证实了抗体在疗效评估中的关键作用。

3. **"CRISPR-Cas9 editing of the DMD gene in human muscle cells: Restoration of dystrophin expression confirmed by antibody staining"**

*作者:Moretti A, et al.*

**摘要**:通过CRISPR基因编辑技术修复DMD基因突变后,使用抗dystrophin抗体进行免疫荧光验证,证实了蛋白表达的恢复。

4. **"Immunohistochemical analysis of dystrophin in muscle biopsies: A diagnostic tool for muscular dystrophies"**

*作者:Mendell JR, et al.*

**摘要**:研究评估了不同抗dystrophin抗体在肌肉活检样本中的诊断价值,为DMD和其他肌营养不良症的鉴别提供标准化方案。

(注:以上文献标题及摘要为示例性内容,实际文献需根据具体研究补充。)

背景信息

Duchenne muscular dystrophy (DMD) is a severe genetic disorder caused by mutations in the DMD gene, which encodes dystrophin, a critical protein for maintaining muscle cell membrane integrity. The absence or dysfunction of dystrophin leads to progressive muscle degeneration, weakness, and premature mortality. DMD antibodies are essential tools in research and diagnostics, specifically designed to detect dystrophin or related proteins in biological samples. These antibodies enable the visualization and quantification of dystrophin expression through techniques like Western blot, immunohistochemistry, and immunofluorescence, aiding in disease diagnosis, mechanistic studies, and therapeutic development.

Monoclonal and polyclonal DMD antibodies target specific epitopes of dystrophin or its isoforms, which are valuable for distinguishing between full-length dystrophin and truncated variants (e.g., in Becker muscular dystrophy). They also help evaluate experimental therapies, such as exon-skipping drugs, gene therapy, or CRISPR-based corrections, by assessing dystrophin restoration in preclinical models or patient-derived cells. Additionally, some antibodies detect utrophin, a dystrophin homolog upregulated in DMD as a compensatory mechanism, providing insights into disease progression and potential therapeutic targets. Commercially available DMD antibodies are rigorously validated for specificity, supporting both basic research and clinical applications aimed at mitigating this devastating condition.

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