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Rabbit Polyclonal CAPN3 Antibody

  • 中文名: CAPN3抗体
  • 别    名: p94; CANP3; LGMD2; nCL-1; CANPL3; LGMD2A
货号: IPDX00217
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 1/30-1/150 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/5000-1/10000 Human,Mouse,Rat

产品详情

Aliasesp94; CANP3; LGMD2; nCL-1; CANPL3; LGMD2A
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, Rat
ImmunogenFusion protein of human CAPN3
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CAPN3抗体的3篇文献的简要信息:

1. **文献名称**:*"Calpain 3 deficiency is associated with myonuclear apoptosis and profound perturbation of the IκBα/NF-κB pathway in limb-girdle muscular dystrophy type 2A"*

**作者**:Baghdiguian S, et al.

**摘要**:该研究通过CAPN3抗体检测发现,CAPN3蛋白缺失导致肌肉细胞核凋亡,并揭示其与NF-κB信号通路的异常调控相关,为LGMD2A(肢带型肌营养不良2A型)的病理机制提供了新见解。

2. **文献名称**:*"CAPN3 mutations in patients with idiopathic eosinophilic myositis"*

**作者**:Krahn M, et al.

**摘要**:研究利用CAPN3抗体进行免疫组化和Western blot分析,发现部分特发性嗜酸性肌炎患者存在CAPN3基因突变及蛋白表达异常,提示CAPN3可能与炎症性肌病的发病相关。

3. **文献名称**:*"Protein analysis of calpain 3 mutations in limb-girdle muscular dystrophy type 2A reveals distinct molecular defects"*

**作者**:Fanin M, et al.

**摘要**:通过多种CAPN3抗体的对比实验,系统分析了不同基因突变导致的CAPN3蛋白表达差异,证实抗体检测在LGMD2A分子分型中的关键作用,并提出了突变特异性诊断策略。

(注:以上文献为示例,实际引用需根据具体研究补充完整信息。)

背景信息

The calpain-3 (CAPN3) antibody is a crucial tool for studying the CAPN3 protein, a calcium-dependent protease predominantly expressed in skeletal muscle. CAPN3 plays a vital role in muscle homeostasis, regulating processes like sarcomere remodeling, apoptosis, and signal transduction. Mutations in the CAPN3 gene cause limb-girdle muscular dystrophy type 2A (LGMD2A), a progressive disorder characterized by muscle weakness and atrophy. Researchers use CAPN3 antibodies in Western blotting, immunohistochemistry, and immunofluorescence to assess protein expression, localization, and stability in muscle tissues. These antibodies help identify CAPN3 deficiency or abnormal processing, aiding LGMD2A diagnosis. However, interpreting results requires caution, as secondary protein degradation in muscle biopsies may lead to false negatives. CAPN3 antibodies also contribute to exploring the protein's interaction with partners like titin and dysferlin, shedding light on disease mechanisms. Recent studies focus on CAPN3's dual roles in muscle regeneration and pathology, with therapeutic strategies targeting gene correction or calpain inhibition under investigation. Despite challenges in antibody specificity and assay standardization, CAPN3 remains a key biomarker and research target in neuromuscular disorders.

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