Profiling of age-related changes in the tibialis anterior muscle proteome of the mdx mouse model of dystrophinopathy.

ABSTRACT: X-linked muscular dystrophy is a highly progressive disease of childhood and characterized by primary genetic abnormalities in the dystrophin gene. Senescent mdx specimens were used for a large-scale survey of potential age-related alterations in the dystrophic phenotype, because the established mdx animal model of dystrophinopathy exhibits progressive deterioration of muscle tissue with age. Since the mdx tibialis anterior muscle is a frequently used model system in muscular dystrophy research, we employed this particular muscle to determine global changes in the dystrophic skeletal muscle proteome. The comparison of mdx mice aged 8 weeks versus 22 months by mass-spectrometry-based proteomics revealed altered expression levels in 8 distinct protein species. Increased levels were shown for

SUBMITTER: Carberry S 

PROVIDER: S-EPMC3471022 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature