Research Interest:
Our laboratory studies the molecular mechanisms underlying muscular dystrophy and associated cardiomyopathy, in particular, with an emphasis on how the sarcolemma (the plasma membrane of muscle cells) maintains its stability and integrity during mechanical stress. It has been recognized that the sarcolemma easily loses its integrity in a large number of muscular dystrophies. However, exactly how the sarcolemma stability and integrity is maintained in striated muscles has not been fully understood. To this point, our findings have elucidated that dystroglycan-linked basal lamina mechanically strengthens the sarcolemmal stability, while dysferlin-mediated membrane repair restores membrane integrity upon injury. Moreover, our studies have established a potential link between the loss of membrane integrity and innate immune activation in vivo. Our long-term goal is to not only better dissect the function and mechanisms by which the membrane stability and integrity is maintained in healthy muscles and disrupted in muscle disorders, but to also determine whether the membrane repair mechanism can be therapeutically targeted for the treatment of these diseases.