Molecular determinants of permeability/gating properties investigated in GJ channels and hemichannels of any connexin isoform, including Cx32, are still debated (Peracchia, 2004; Zhou et al., 2009; Oh and Bargiello, 2015), but their elucidation could help to answer why different Cx32 mutations cause a similar phenotype equivalent to a loss-of-function in the PNS of CMT1X patients. Here, GJB1 is linked to X-linked Charcot-Marie-Tooth disease type 1.