Most interestingly, we show that treatment with riluzole, an FDA-approved drug for amyotrophic lateral sclerosis (ALS) treatment [31, 32], partly corrects the defects in Pol III assembly in the presence of a POLR3B subunit having the R103H substitution known to be causative for POLR3-related leukodystrophy. This evidence concerns the gene POLR3B and leukodystrophy.