Data from a cellular model of polyglutamine disease indicate that phosphorylation of eIF2 alpha by PERK mediates the induction of LC3-II [45], while a recent study in cellular and mouse models of superoxide dismutase 1 (SOD1)-linked ALS show that XBP1 deletion activates autophagy [44]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.