Hughes MC, Ramos SV, Turnbull PC, et al. Impairments in left ventricular mitochondrial bioenergetics precede overt cardiac dysfunction and remodelling in Duchenne muscular dystrophy. J Physiol. 2020;598(7):1377-1392. doi:10.1113/JP277306
Significance of the research:
98% of patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy, with 40% developing heart failure. Using mice, the study by Hughes and Ramos et al found that the energy-producing parts of cells known as ‘mitochondria’ begin to fail at very early stages of this disease before the hearts become dysfunctional. This discovery suggests that mitochondria may be a therapeutic target for the treatment of cardiomyopathy in DMD.