Additionally, expression of mutant GlyRS exclusively in the cytosol is sufficient to trigger peripheral neuropathy in Drosophila (Ermanoska et al., 2014) and mice (Lee et al., 2014; Seo et al., 2014a,b), suggesting that mitochondrial dysfunction is not the primary cause of CMT2D neuropathy. Here, GARS1 is linked to Charcot-Marie-Tooth disease type 2D.