Overexpression of mutant GlyRS on the background of active endogenous GlyRS in Drosophila induces a CMT2D phenotype (Ermanoska et al., 2014), and expression of the Gars1C201R and Gars1P278KY variants in mice show dose-dependent toxicity, which cannot be counteracted with overexpression of wildtype GlyRS (Motley et al., 2011). Here, GARS1 is linked to Autosomal dominant Charcot-Marie-Tooth disease type 2D.