Two mouse models of CMT2D that share pathological features with the human disease, with differing severity, are caused by dominant amino acid substitutions in Gars. The GarsNmf249 allele (hereafter abbreviated Nmf249) causes reduced body weight and impaired mobility in heterozygous mice [25]. This evidence concerns the gene GARS1 and Autosomal dominant Charcot-Marie-Tooth disease type 2D.