Muscle dystrophy in Caenorhabditis elegans, Roy, Sarath; Brandow, Christopher
College of Science and Mathematics
Professor: Dr. Rachel Hopp
Caenorhabditis elegan’s dys-1, a homolog of human (Duchenne Muscle Dystrophy) DMD dystrophin gene, is a dystrophin like gene that when mutated leads to hyperactivity and hypercontractivity of muscles in animals. DMD is an X-linked muscle disease and according to previous studies many of these mutations can be corrected at the posttranscriptional level by alternative splicing which removes the mutated exon. Research in this field can provide an indisputable prospect into correction by alternative splicing and for further gene therapy. Dystrophin is essential for assembly of the dystrophin-glycoprotein complex and provides a strong connection between the cytoskeleton and the extracellular matrix. RNA interference, RNAi, is used to induce the specific knockdown of dys-1 gene. We received a double stranded RNAi feeding strain (targeting dys-1) from the Dolan DNA Learning Center at the Cold spring Harbor Laboratories. A dys-1 knockdown in C. elegans is expected to exhibit hyperactivity and hypercontractivity of muscular functions. In this experiment, features of the dys-1 knockdown nematodes are observed by placing the worms on plates that contain a bacteria food source only around the outer edge. Worms were placed in the center of each plate and their movements and speed were observed using a dissecting microscope. Photographs and video recordings of both control and dys-1 knockdown worms were obtained via iPhone and analyzed to visualize the hyperactivity in the knockdowns as compared to the wild type. Data analysis was done as distance over time as they moved from the center to the outer edges. Since dys-1 knockdown worms are expected to be more hyperactive in their movement, the video recordings should display the movement difference when compared to the wild type in that mutated worms had a hyper body movement or what appears as an uncoordinated twitch. Results will be presented at the symposium.
