Murine models of the common human DNM2 R465W dominant CNM mutation do replicate aspects of the human phenotype, and not unexpected considering the close links between endocytic and autophagic pathways, show variable abnormalities of autophagy: Durieux et al. (2010b, 2012) demonstrated a slowly progressive myopathy with upregulation of genes involved in ubiquitin–proteosome (UPS) and autophagy pathways in a heterozygous knock-in mouse model of the common CMT-associated heterozygous DNM2 mutation R465W. Here, DNM2 is linked to Charcot-Marie-Tooth disease.