The diurnal regulation of muscle core clock and mitochondrial quality control expression was examined in 18 dystrophin-deficient C57BL/10ScSn-Dmdmdx (mdx) mice, an established model of DMD every 4 hours beginning at the dark cycle.
‘Impaired oxidative capacity and mitochondrial function contribute to the dystrophic pathology in muscles of Duchenne muscular dystrophy (DMD) patients.’
It was found that throughout the entire light-dark cycle, extensor digitorum longus (EDL) muscles from mdx mice had decreased core clock mRNA expression and disrupted mitochondrial quality control mRNA expression. The expression of proteins involved in mitochondrial biogenesis and autophagy/mitophagy were also dysregulated in tibialis anterior muscles of mdx mice.
These findings suggest that dystrophin deficiency in mdx mice impairs the regulation of the core clock and mitochondrial quality control, with relevance to DMD and related disorders.
Source: Medindia
