Respiratory pathology in the mdx/utrn -/- mouse: A murine model for Duchenne Muscular Dystrophy (DMD).

ABSTRACT: Duchenne muscular dystrophy (DMD) is an X-linked devastating disease caused by a lack of dystrophin which results in progressive muscle weakness. As muscle weakness progresses, respiratory insufficiency and hypoventilation result in significant morbidity and mortality. The most studied DMD mouse model- the mdx mouse- has a milder respiratory phenotype compared to humans, likely due to compensatory overexpression of utrophin. mdx/utrn-/- mice lack both dystrophin and utrophin proteins. These mice have an early onset of muscular dystrophy, severe muscle weakness, and premature death, but the respiratory pathophysiology is unclear. The objective of this study is to characterize the respiratory pathophysiology and histopathology using whole body plethysmography to measure breathing and metabol

SUBMITTER: Hernandez Rodriguez MY 

PROVIDER: S-EPMC11805407 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature