Myotilin Antibody (B-3): sc-393958

Myotilin Antibody (B-3) is a mouse monoclonal IgG2a antibody that detects myotilin in human samples through applications such as western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Myotilin is a crucial sarcomeric protein encoded by a gene located on human chromosome 5q31, playing a significant role in muscle structure and function. Anti-myotilin antibody (B-3) is primarily localized in the Z-line of myofibrils, where myotilin binds to α-actinin, facilitating the structural integrity of the sarcomere. This interaction is vital for maintaining muscle contraction and overall muscle health. Defects in the myotilin gene have been linked to autosomal dominant limb-girdle muscular dystrophy (LGMD), a condition characterized by progressive weakness in the hip and shoulder girdles, along with distinctive speech patterns. Affected individuals exhibit degeneration of myofibers, variations in fiber size, fiber splitting, centrally located myonuclei, and an increased number of autophagic vesicles, highlighting the critical role of myotilin in muscle integrity and function. Myotilin monoclonal antibody (B-3) is an essential tool for researchers studying muscle diseases and the underlying mechanisms of myofibrillar organization.

The preferred Myotilin antibody is Myotilin Antibody (E-10): sc-393957.