The second most prevalent autosomal dominant hereditary demyelinating neuropathy is Charcot–Marie–Tooth disease type 1B (CMT1B), caused by activation of UPR components coupled with accumulation of mutant protein myelin protein Zero (P0, MPZ), as a consequence of ER stress (Santoro et al., 2004; Khajavi et al., 2005; Saporta et al., 2011). Here, MPZ is linked to Charcot-Marie-Tooth disease type 1B.