Progressive Proteome Changes in the Myocardium of a Pig Model for Duchenne Muscular Dystrophy.

ABSTRACT: Duchenne muscular dystrophy (DMD), caused by mutations in the dystrophin gene, is characterized by progressive muscle weakness. Even though DMD manifests first in skeletal muscle, heart failure is a major cause of death in late-stage DMD. To get insights into DMD-associated cardiomyopathy, we performed a proteome analysis of myocardium from a genetically engineered porcine DMD model resembling clinical and pathological hallmarks of human DMD. To capture DMD progression, samples from 2-day- and 3-month-old animals were analyzed. Dystrophin was absent in all DMD samples, and components of the dystrophin-associated protein complex were decreased, suggesting destabilization of the cardiomyocyte plasma membrane and impaired cellular signaling. Furthermore, abundance alterations of proteins know

SUBMITTER: Tamiyakul H 

PROVIDER: S-EPMC7495112 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature