Pathogenic variants of genes encoding aminoacyl-tRNA synthetases (ARS) have been linked to a number of neurodegenerative diseases, including hypomyelinating leukodystrophies (e.g., EPRS1 [17], VARS1 [18], DARS1 [28] and RARS1 [29]). Here, EPRS1 is linked to leukodystrophy.