ERCC8 and Cowden syndrome 1: Inactivation ofGG-NER by an XPC knockout in CSBm/m mice leads to a similar phenotype,with dysmyelination, slow development, and low body weight: characteristics thatresemble those of patients with CS (Laposaet al., 2007; vander Pluijm et al., 2007; Revet et al., 2012).CSA-/-/XPA-/- mice also show evidence of neurologicalproblems, life expectancy reduced to ~1 month, an increase in cellular oxidativephosphorylation, and lipodystrophy (Brace etal., 2013, 2016).