Furthermore, they show that the C-terminus of XPG could play a role in the CS symptoms, and that the XPG-deficient Xpg−/− mice may reproduce the phenotype of Xpa−/−Csb−/−, Xpc−/−Csb−/− or Xpa−/−Csa−/− double mutant mice [62], [66]–[69]. Here, XPA is linked to Cowden syndrome 1.