ALS-associated mutations in C9ORF72, SOD1 (G93A), TARDBP, TBK1, FUS (P525L), FIG4, OPTN, UBLN2, SQSTM1, CHMP2B, and ALS2 dysregulate macroautophagy and dysregulated macroautophagy is linked to neurodegeneration in ALS [101,102]. This evidence concerns the gene CHMP2B and amyotrophic lateral sclerosis.