Biomarkers for Duchenne muscular dystrophy progression: impact of age in the mdx tongue spared muscle.

ABSTRACT:

Background

Duchenne muscular dystrophy (DMD) is a severe form of muscular dystrophy without an effective treatment, caused by mutations in the DMD gene, leading to the absence of dystrophin. DMD results in muscle weakness, loss of ambulation, and death at an early age. Metabolomics studies in mdx mice, the most used model for DMD, reveal changes in metabolites associated with muscle degeneration and aging. In DMD, the tongue muscles exhibit unique behavior, initially showing partial protection against inflammation but later experiencing fibrosis and loss of muscle fibers. Certain metabolites and proteins, like TNF-α and TGF-β, are potential biomarkers for dystrophic muscle characterization.

Methods

To investigate disease progression and aging, we utilized young (1 month old)

SUBMITTER: Lorena MDSV 

PROVIDER: S-EPMC10500803 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature