Accumulation of misfolded proteins and activation of stress response in Schwann cells is thought to be at the basis of different forms of demyelinating CMT, such as the AD CMT1E due to point mutations in the PMP22 gene and the AD CMT1B, associated with mutations in the MPZ (myelin protein zero) [86, 99–101]. This evidence concerns the gene MPZ and Charcot-Marie-Tooth disease type 1B.