Conditional deletion of XBP1 in the central nervous system (CNS) provides protective effects through upregulation of autophagy levels, improving motor performance in ALS, PD and Huntington's disease models (35, 37, 41), whereas XBP1 deficiency does not affect Prion pathogenesis in vivo (42). Here, XBP1 is linked to amyotrophic lateral sclerosis.