We examined neuronal proteotoxicity with a well-characterized expanded polyglutamine (polyQ, with 128Q) model in C. elegans touch receptor neurons (Parker et al., 2001, 2005) and with a worm strain that expresses full length human mutant TDP-43[A315T] (mTDP-43), a mutation linked to ALS (Kabashi et al., 2008) in C. elegans GABAergic motor neurons (Vaccaro et al., 2012). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.