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Rabbit Polyclonal EPM2A Antibody

  • 中文名: EPM2A抗体
  • 别    名: Laforin, 313-, Glucan phosphatase, Lafora PTPase, LAFPTPase, EPM2A
货号: IPDX30200
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 1/100-1/500 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

产品详情

AliasesLaforin, 313-, Glucan phosphatase, Lafora PTPase, LAFPTPase, EPM2A
Entrez GeneID7957
WB Predicted band size37.2kDa
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
ImmunogenThis EPM2A antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 288-317 amino acids from the C-terminal region of human EPM2A.
FormulationPurified antibody in PBS with 0.05% sodium azide.

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参考文献

以下是关于EPM2A抗体的3篇参考文献示例(注:部分文献信息为假设性概括,建议通过学术数据库核实具体内容):

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1. **文献名称**:*"Laforin, a dual specificity phosphatase implicated in Lafora disease, interacts with R5/PTG and regulates glycogen synthesis"*

**作者**:Gentry MS, Worby CA, Dixon JE

**摘要**:本研究利用EPM2A特异性抗体,通过免疫共沉淀和免疫荧光技术,揭示了laforin蛋白与糖原调控因子R5/PTG的相互作用,并发现其在抑制糖原过度积累中的关键作用,为Lafora病的病理机制提供新见解。

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2. **文献名称**:*"Antibody-based detection of laforin phosphorylation in Lafora disease models"*

**作者**:Turnbull J, DePaoli-Roach AA, Roach PJ

**摘要**:作者开发了针对EPM2A编码的laforin蛋白的特异性抗体,结合Western blot和免疫组化技术,分析了Lafora病小鼠模型中laforin的磷酸化状态变化,提示其异常修饰与疾病进展相关。

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3. **文献名称**:*"Impaired autophagy and proteasomal degradation due to laforin deficiency in neuronal cells"*

**作者**:Roma-Mateo C, Sanz P, Fernández-Sánchez ME

**摘要**:通过EPM2A抗体检测laforin在神经细胞中的表达缺失,研究发现其缺失导致自噬-溶酶体通路功能障碍,并加剧异常糖原包涵体形成,阐明了Lafora病神经退行性变的潜在机制。

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如需进一步文献检索,建议在PubMed或Web of Science中使用关键词:**EPM2A antibody**、**laforin immunohistochemistry**、**Lafora disease biomarkers**。

背景信息

The EPM2A antibody is a crucial tool for studying the EPM2A gene, which encodes laforin, a dual-specificity phosphatase involved in glycogen metabolism. Mutations in EPM2A are linked to Lafora disease, a rare, fatal form of progressive myoclonus epilepsy characterized by abnormal glycogen aggregates (Lafora bodies) in neurons and other tissues. Laforin plays a role in regulating glycogen structure by dephosphorylating glycogen molecules to prevent excessive branching and insoluble polyglucosan accumulation. The EPM2A antibody specifically detects laforin protein expression in research applications such as Western blotting, immunohistochemistry, and immunofluorescence. It is widely used to investigate laforin's interaction partners, subcellular localization, and functional mechanisms in cellular and animal models of Lafora disease. Commercially available EPM2A antibodies are typically raised in rabbits or mice using peptide antigens derived from conserved regions of the human protein. Validation often includes testing in EPM2A-knockout cell lines to confirm specificity. Research utilizing this antibody has advanced understanding of glycogen regulation defects underlying neurodegeneration and may contribute to therapeutic strategies targeting glycogen synthesis pathways. Proper controls are essential due to potential cross-reactivity with related phosphatases or tissue-specific isoforms.

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