Anti-MYOT / Myotilin

Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml. MYOT antibody detects Myotilin, a structural protein of the sarcomeric Z-disc that maintains muscle integrity and organization. Encoded by the MYOT gene on chromosome 5q31.2, this cytoskeletal protein binds alpha-actinin, filamin C, and other Z-disc components to stabilize actin filament cross-linking and support mechanical tension during muscle contraction. Myotilin plays a central role in maintaining sarcomere alignment, muscle elasticity, and myofibrillar assembly.Structurally, Myotilin is a 57 kilodalton cytoplasmic protein containing two immunoglobulin-like domains at its C-terminus that mediate interactions with alpha-actinin and filamin. Its N-terminal serine-rich region supports actin binding and cross-linking, anchoring thin filaments at the Z-disc. Myotilin is expressed predominantly in skeletal and cardiac muscle, where it acts as a scaffold linking actin filaments to structural and signaling molecules that maintain contractile function.The MYOT antibody is widely used in muscle biology, myopathy, and cytoskeletal research to study sarcomere structure, actin filament organization, and myofibrillogenesis. Western blot analysis identifies a 57 kilodalton band corresponding to Myotilin, while immunohistochemistry shows distinct Z-disc staining in muscle fibers. This antibody supports investigations into muscle development, maintenance, and degenerative conditions affecting the sarcomere.Mutations in MYOT cause various myofibrillar myopathies, including limb-girdle muscular dystrophy type 1A and spheroid body myopathy. These mutations lead to Myotilin aggregation, sarcomeric disorganization, and progressive muscle weakness. Altered MYOT expression has also been linked to cardiac remodeling and hypertrophy. The MYOT antibody provides a critical tool for examining these pathogenic mechanisms and evaluating Myotilin's interactions with actin-binding and signaling proteins. NSJ Bioreagents validates this antibody for its applications, ensuring dependable results for muscle and cytoskeletal studies.