Moreover, administration of sephin1, an inhibitor of GADD34, was effective in two models of neurodegenerative diseases in mice that are associated with ER stress—Charcot–Marie–Tooth 1B (CMT-1B) and SOD1-ALS (49)—and administration of raphin-1 to mouse models of Huntington disease harboring the HD82Q mutation improved their weight and decreased the SDS-insoluble huntingtin and nuclear assemblies in their cortices. Here, SOD1 is linked to juvenile Huntington disease.