Here we show that the small-molecule IFB-088 is able to readjust protein homeostasis and to ameliorate disease features in two models of demyelinating CMT1, the MpzR98C/ + (CMT1B) and the C3-PMP22 (CMT1A) mice. This evidence concerns the gene PMP22 and Charcot-Marie-Tooth disease type 1B.