Trehalose has been proved active against aggregation of polyQ-expanded huntingtin (43,44), beta-amyloid (36,42,56), polyA-binding protein nuclear 1 (PABPN1) (45), the pathological prion protein (46), the amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD)-associated protein TDP-43 (57), mutant synuclein (47) and also on the ARpolyQ (37). This evidence concerns the gene PABPN1 and amyotrophic lateral sclerosis.