Current Research and Scholarly Interests
Duchenne muscular dystrophy (DMD) is an X-chromosome-linked genetic disease that is caused by a mutation in the dystrophin gene and affects 1 in every 3500 boys. DMD patients suffer progressive muscle wasting and eventual cardiorespiratory failure that results in an early death in the second or third decade of life. Although extensive research effort has been invested, lack of a good mouse model that mimics the cardiac failure hinders research. We have developed a novel mouse model that exhibit all the symptoms found in DMD patients and our research is aimed at understanding the cardiac failure in DMD for future therapeutic interventions. Our mouse model fully recapitulates the DMD symptoms because we also took into account of the size of human protection DNA on chromosomal ends (telomere) compared to mouse. We would like to study the cause of cardiac failure in our mouse model by 1) determine if telomere shortening is specific to cardiomyocytes, 2) evaluate the level of cellular damage caused by oxidative stress and 3) identify the source of oxidative stress. These experiments will help us to better understand cardiac failure in DMD patients and allow testing of therapeutic interventions.