Dystrophin deficiency disrupts muscle clock expression and mitochondrial quality control in mdx mice.
Justin P Hardee, Marissa K Caldow, Audrey SM Chan, Stuart K Plenderleith, Jennifer Trieu, René Koopman, Gordon S Lynch
Am J Physiol Cell Physiol | Published : 2021
Impaired oxidative capacity and mitochondrial function contribute to the dystrophic pathology in muscles of patients with Duchenne muscular dystrophy (DMD) and in relevant mouse models of the disease. Emerging evidence suggests an association between disrupted core clock expression and mitochondrial quality control, but this has not been established in muscles lacking dystrophin. We examined the diurnal regulation of muscle core clock and mitochondrial quality control expression in dystrophin-deficient C57BL/10ScSn-Dmdmdx (mdx) mice, an established model of DMD. Male C57BL/10 (BL/10; n = 18) and mdx mice (n = 18) were examined every 4 h beginning at the dark cycle. Throughout the entire ligh..View full abstract
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Awarded by Department of Health, Australian Government | National Health and Medical Research Council (NHMRC)