ERCC6 and Cowden syndrome 1: The defects in LMNA, WRN and ERCC6/8 seen in HGPS, WS and CS, respectively, all cause major instability of the nuclear envelope and large scale unrepaired genomic damage, resulting in major curtailment of average lifespan to 14.5 years for HGPS and 54 years for WS [3, 5, 10, 11].