The Effect of Age on the Spatial Variation in the Microstructure of Healthy and Dystrophic Diaphragm Muscle
University of Virginia
Currently, there are no treatments that address the root cause of the most common type of muscular dystrophy, Duchenne muscular dystrophy (DMD). DMD affects 1 in 3,500 male births and results from an x-linked gene mutation that causes a deficiency of the protein dystrophin. A DMD patient typically dies in his early twenties by respiratory failure caused by progressive fibrosis and muscle degeneration of the diaphragm. A healthy functioning diaphragm has spatially varying structure and this heterogeneity is diminished in dystrophic diaphragms. While age studies on healthy and dystrophic diaphragms have been completed, none have examined the spatial variability of its microstructure. The purpose of this research is to evaluate the effects of aging on regional tissue structure, inflammatory status, and vascularity in healthy diaphragms as compared to dystrophic diaphragms using a mouse model of disease. Understanding the maladaptations of diaphragm muscle caused by DMD as subjects' age could eventually lead to the creation of more effective, localized, and specific treatments for the disease.