The iPSC-derived motor neurons show a reduction in the LC3-positive vesicles (Haidar et al. 2019), and accumulation of LC3IIB and SQSTM1/p62 is detected in the motor axons of the HSPB8 knock-in model (Bouhy et al. 2018), suggesting autophagy as a common defective mechanism in CMT2/distal HMN caused by sHSP mutations. The gene discussed is SQSTM1; the disease is Charcot-Marie-Tooth disease type 2.