EPM2A抗体

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     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P09805(EPM2A Antibody) at dilution 1/55. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P09805(EPM2A Antibody) at dilution 1/55. (Original magnification: ×200)    

  
  

The EPM2A antibody is a crucial tool in studying Lafora disease, a rare, fatal autosomal recessive form of progressive myoclonic epilepsy caused by mutations in the EPM2A gene. This gene encodes laforin, a dual-specificity phosphatase involved in glycogen metabolism regulation. Laforin interacts with malin (encoded by EPM2B) to prevent abnormal glycogen aggregation by dephosphorylating glycogen and promoting its proper branching. Defects in this process lead to toxic, insoluble polyglucosan inclusions (Lafora bodies) in neurons and other tissues, driving neurodegeneration and seizures.

EPM2A antibodies are primarily used to detect laforin expression in research settings, aiding in mechanistic studies of Lafora disease pathogenesis. They enable techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess protein levels, localization, and interactions in cellular or animal models. Commercial EPM2A antibodies are typically raised against specific epitopes of human laforin, though cross-reactivity with orthologs in model organisms (e.g., mice) is common.

Recent studies highlight their utility in exploring therapeutic strategies, such as gene therapy or enzyme replacement, and in validating disease models. Challenges include ensuring antibody specificity, as laforin's structural homology with other phosphatases may cause cross-reactivity. Ongoing research using these antibodies continues to unravel laforin’s roles beyond glycogen regulation, including autophagy and proteostasis, offering insights into broader neurodegenerative mechanisms.