Postnatal growth failure, short life span, and early onset of cellular senescence and subsequent immortalization in mice lacking the xeroderma pigmentosum group G gene.

摘要: The xeroderma pigmentosum group G (XP-G) gene (XPG) encodes a structure-specific DNA endonuclease that functions in nucleotide excision repair (NER). XP-G patients show various symptoms, ranging from mild cutaneous abnormalities to severe dermatological impairments. In some cases, exhibit growth failure and life-shortening neurological dysfunctions, which are characteristics of Cockayne syndrome (CS). known XPG protein function as the 3' nuclease NER, however, cannot explain development CS certain patients. To gain an insight into protein, we have generated examined mice lacking xpg (the mouse counterpart human gene) alleles. xpg-deficient exhibited postnatal underwent premature death. Since XPA-deficient mice, totally defective do not such our data indicate performs additional function(s) besides its role NER. Our vitro studies showed primary embryonic fibroblasts isolated senescence early onset immortalization accumulation p53.