Integrin dysregulation as a possible driver of matrix remodeling in Laminin-deficient congenital muscular dystrophy (MDC1A)

Abstract

Our results suggest that overexpression of Integrin-αV and -α5 are likely contributing to secondary pathologies in MDC1A. We also believe that downregulation of Integrin-αV could be partially responsible for Losartan's antifibrotic effect and therefore could serve as a novel therapeutic target in MDC1A and other degenerative fibrotic diseases.