Aminoacyl-tRNA synthetases (aaRSs) are the most represented protein family associated with CMT to date, with dominant mutations described in six genes (Figure 1, Table 1): glycyl-RS (GARS1) [18], tyrosyl-RS (YARS1) [19], alanyl-RS (AARS1) [20], histidyl-RS (HARS1) [21], methionyl-RS (MARS1) [22] and tryptophanyl-RS (WARS1) [23]. The gene discussed is AARS1; the disease is Charcot-Marie-Tooth disease.