Recently, Kagiava et al. and Scherer et al. identified that Cx32 mutations Cx32 p.Arg75Trp, p.Asn175Asp, and p.Arg142Trp exhibit dominant negative effects on WT Cx32 in vivo and no significant improvement of peripheral neuropathy caused by these mutations was achieved through intrathecal gene therapy [176,180,181,189]. Here, GJB1 is linked to peripheral neuropathy.