These NER nuclease–deficient models have severalneurodegenerative features, altered metabolism, and a reduced lifespan (Weeda et al., 1997; Barnhoorn et al., 2014).Notably, despite the lipodystrophy observed in all these animal models, dietaryrestriction and methionine restriction (which are metabolic interventions known toincrease the lifespan of several species) reversed some of the neurodegenerationindicators of the CSA-/-/XPA-/- and ERCC1Δ/-models, with dietary restriction nearly doubling the lifespan of ERCC1Δ/-mice. The gene discussed is XPA; the disease is lipodystrophy.