Mechanical and non-mechanical functions of Dystrophin can prevent cardiac abnormalities in Drosophila.

ABSTRACT: Dystrophin-deficiency causes cardiomyopathies and shortens the life expectancy of Duchenne and Becker muscular dystrophy patients. Restoring Dystrophin expression in the heart by gene transfer is a promising avenue to explore as a therapy. Truncated Dystrophin gene constructs have been engineered and shown to alleviate dystrophic skeletal muscle disease, but their potential in preventing the development of cardiomyopathy is not fully understood. In the present study, we found that either the mechanical or the signaling functions of Dystrophin were able to reduce the dilated heart phenotype of Dystrophin mutants in a Drosophila model. Our data suggest that Dystrophin retains some function in fly cardiomyocytes in the absence of a predicted mechanical link to the cytoskeleton. Interestingly,

SUBMITTER: Taghli-Lamallem O 

PROVIDER: S-EPMC4340692 | biostudies-literature | 2014 Jan

REPOSITORIES: biostudies-literature