Cardiomyopathy in the dystrophin/utrophin-deficient mouse model of severe muscular dystrophy is characterized by dysregulation of matrix metalloproteinases
DA Delfín, KE Zang, KE Schill, NT Patel… - Neuromuscular …, 2012 - Elsevier
Cardiomyopathy is a significant component in Duchenne muscular dystrophy. Although mdx
mice are deficient in dystrophin, they only develop mild indicators of cardiomyopathy before
1year-of-age, making therapeutic investigations using this model lengthy. In contrast, mdx
mice also lacking utrophin (utrn−/−; mdx) show severely reduced cardiac contractile function
and histological indicators of cardiomyopathy by 8–10weeks-of-age. Here we demonstrate
that utrn−/−; mdx mice show a similar pattern of cardiac damage to that in dystrophic …
mice are deficient in dystrophin, they only develop mild indicators of cardiomyopathy before
1year-of-age, making therapeutic investigations using this model lengthy. In contrast, mdx
mice also lacking utrophin (utrn−/−; mdx) show severely reduced cardiac contractile function
and histological indicators of cardiomyopathy by 8–10weeks-of-age. Here we demonstrate
that utrn−/−; mdx mice show a similar pattern of cardiac damage to that in dystrophic …