Duchenne's Muscular Dystrophy (DMD) is caused by a deficiency in dystrophin protein. DMD is distinguishable through muscle degeneration and weakness. Dystrophin protein is a necessary structural link between the sarcolemma and the cytoskeleton. Studies show Neuronal Nitric Oxide Synthase (nNOS), a critical enzyme in the sarcolemma, that catalyzes nitric oxide (NO), is a molecular component of the Dystrophin-Glycoprotein Complex (DGC). To rescue cells from the effects of a dystrophin deficiency, we will expose the muscle sarcolemma to NO by using gas plasma. Three methods will be tested: 1) treatment with air through a plasma device, as our control, 2) treatment with NO through the plasma device, and 3) treatment with NO via Cold Atmospheric Pressure Source (CAPS) to generate a NO plasma. Q-RT-PCR analysis and confocal microscopy will allow quantification of DGC stability at the plasma membrane. We propose to answer mechanistic questions such as: 1) does an increase in NO levels affect the expression of muscle specific genes in the presence and absence of dystrophin, 2) will increased levels of NO stabilize the DGC within the cell, and 3) are other types of muscle cells (skeletal, cardiac, and smooth) affected by increasing NO in cells. Thus, we predict NO treatment will rescue the deficiency in absence of dystrophin.
Acknowledgment of Support: Research was supported by a Grant-in-Aid of Research administered by Sigma Xi, The Scientific Research Society
Compton, Jennica; Karriem, Lynn; and Blomquist, AnnaGrace, "Dystrophin-Glycoprotein Complex and Reactive Oxygen Species" (2017). 2017 Undergraduate Research and Scholarship Conference.