GJB2 and keratoderma hereditarium mutilans: We showed here that indeed epidermal expression of Cx26S17F produced the severe PPK seen in KIDS, as well as a short, blunted tail reminiscent of autoamputated digits in patients with the Cx26-linked Vohwinkel Syndrome.35 Furthermore, Cx26CK14-S17F/+ mice in this study were generally healthy suggesting that expression of the S17F mutant in other tissues such as liver, kidney, brain, and gut may have contributed to reduced viability seen in mice with global expression of the S17F mutant.