Haploinsufficiency of utrophin gene worsens skeletal muscle inflammation and fibrosis in mdx mice

L Zhou, JA Rafael-Fortney, P Huang, XS Zhao… - Journal of the …, 2008 - Elsevier
To address whether mdx mice with haploinsufficiency of utrophin (mdx/utrn+/−) develop
more severe skeletal muscle inflammation and fibrosis than mdx mice, to represent a better
model for Duchenne muscular dystrophy (DMD), we performed qualitative and quantitative
analysis of skeletal muscle inflammation and fibrosis in mdx and mdx/utrn+/− littermates.
Inflammation was significantly worse in mdx/utrn+/− quadriceps at age 3 and 6 months and
in mdx/utrn+/− diaphragm at age 3 but not 6 months. Fibrosis was more severe in …