Early postnatal ataxia and abnormal cerebellar development in mice lacking Xeroderma pigmentosum Group A and Cockayne Syndrome Group B DNA repair genes

Murai, Machiko; Enokido, Yasushi; Inamura, Naoko; Yoshino, Masafumi; Nakatsu, Yoshimichi; van der Horst, Gijsbertus T. J.; Hoeijmakers, Jan H. J.; Tanaka, Kiyoji; Hatanaka, Hiroshi

doi:10.1073/pnas.231329598

Early postnatal ataxia and abnormal cerebellar development in mice lacking Xeroderma pigmentosum Group A and Cockayne Syndrome Group B DNA repair genes

Xeroderma pigmentosum (XP) and Cockayne syndrome (CS) are rare autosomal recessive disorders associated with a defect in the nucleotide excision repair (NER) pathway required for the removal of DNA damage induced by UV light and distorting chemical adducts. Although progressive neurological dysfu…