ERCC1 and Cowden syndrome 1: The occurrence of neurological abnormalities and impaired motor behavior in Xpg−/− mice (Figure 2E and S2), as well as the abundant neurodegenerative features in ERCC1-deficient and combined XP/CS mouse models [89]–[91], prompted us to investigate the central nervous systems of Xpg−/− animals for neurodegenerative changes.