Furthermore, mutations in genes encoding critical proteins of the mitochondrial translation system, including MTO1, GTBP3, and ELAC2 (Baruffini et al., 2013, Haack et al., 2013, Kopajtich et al., 2014) and several mt-aaRS mutations in AARS2, PARS2, SARS2, and YARS2, have been shown to cause hypertrophic cardiomyopathy (Belostotsky et al., 2011, Götz et al., 2011, Riley et al., 2010, Rivera et al., 2013, Shahni et al., 2013, Sofou et al., 2015). Here, AARS1 is linked to hypertrophic cardiomyopathy.