Despite that no protein misfolding‐related pathology had been reported in XP patients or in these transgenic XP mouse models, the neurodegenerative features observed in XPG and ERCC1 models indicate that defects in nucleotide excision repair mechanism alone may lead to neurodegeneration and conformational assessment of aggregation‐prone proteins (e.g., tau, Aβ and α‐synuclein) may be a useful future step in XPG‐ and ERCC1‐deficient mouse models. Here, ERCC1 is linked to xeroderma pigmentosum.