Cockayne syndrome (CS) is a rare autosomal recessive disorder linked to mutations in the ERCC8 and ERCC6 genes encoding for Cockayne syndrome protein A (CSA) and Cockayne syndrome protein B (CSB), respectively (Troelstra et al., 1992; Henning et al., 1995) both of which play a role in TCR (Bregman et al., 1996; Svejstrup, 2003). The gene discussed is ERCC6; the disease is Cowden syndrome 1.