Nucleotide excision repair deficient mouse models and neurological disease

LJ Niedernhofer - DNA repair, 2008 - Elsevier
Nucleotide excision repair (NER) is a highly conserved mechanism to remove helix-
distorting DNA base damage. A major substrate for NER is DNA damage caused by
environmental genotoxins, most notably ultraviolet radiation. Xeroderma pigmentosum,
Cockayne syndrome and trichothiodystrophy are three human diseases caused by inherited
defects in NER. The symptoms and severity of these diseases vary dramatically, ranging
from profound developmental delay to cancer predisposition and accelerated aging. All …