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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Mouse skeletal muscle tissue lysate, Primary antibody: P10188(MYOT Antibody) at dilution 1/900, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 1 second    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P10188(MYOT Antibody) at dilution 1/65. (Original magnification: ×200)    

  
  

MYOT antibodies, also known as anti-myotilin antibodies, are autoantibodies associated with certain autoimmune myopathies, particularly inclusion body myositis (IBM) and limb-girdle muscular dystrophy (LGMD)-type presentations. Myotilin is a sarcomeric protein expressed in skeletal and cardiac muscle, playing a structural role in Z-disc organization and myofibrillar stability. Autoantibodies targeting myotilin are rare and not fully characterized, but their presence often correlates with specific clinical phenotypes, such as muscle weakness, myofibrillar disintegration, and protein aggregates in muscle biopsies.

First identified in patients with myopathies overlapping IBM and LGMD features, MYOT antibodies are detected via immunoprecipitation or immunoassay. Their pathogenic role remains unclear; they may arise secondary to muscle damage or contribute to disease progression through immune-mediated mechanisms. Unlike classic myositis-specific antibodies (e.g., anti-Jo-1), MYOT antibodies lack definitive diagnostic specificity but aid in subclassifying heterogeneous muscle disorders. Research is ongoing to clarify their clinical utility and interaction with genetic myotilin mutations linked to LGMD. Current evidence suggests MYOT antibodies serve as biomarkers for特定 immune-driven myopathies, warranting further investigation into their diagnostic and prognostic relevance.