Autosomal dominant mutations within several ARS are known to cause distal motor neuropathy or polyneuropathies, including cytoplasmic ARS such as alanyl-tRNA synthetase (AARS) [15], tyrosyl-tRNA (YARS) [16] and lysyl-tRNA synthetase (KARS) [17] as well as the bifunctional GARS [3]. The gene discussed is AARS1; the disease is Motor axonal neuropathy.