MICE WITH DNA-REPAIR GENE (ERCC-1) DEFICIENCY HAVE ELEVATED LEVELS OF P53, LIVER NUCLEAR ABNORMALITIES AND DIE BEFORE WEANING

被引：290

作者：

MCWHIR, J

SELFRIDGE, J

HARRISON, DJ

SQUIRES, S

MELTON, DW

机构：

[1] UNIV EDINBURGH,INST CELL & MOLEC BIOL,MAYFIELD RD,EDINBURGH EH9 3JR,MIDLOTHIAN,SCOTLAND

[2] UNIV EDINBURGH,DEPT PATHOL,EDINBURGH EH8 9AG,MIDLOTHIAN,SCOTLAND

[3] UNIV CAMBRIDGE,DEPT ZOOL,CANCER RES CAMPAIGN MAMMALIAN CELL DNA REPAIR RES GRP,CAMBRIDGE CB2 3EJ,ENGLAND

来源：

NATURE GENETICS | 1993年 / 5卷 / 03期

关键词：

D O I：

10.1038/ng1193-217

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

Defects in nucleotide excision repair are associated with the human condition xeroderma pigmentosum which predisposes to skin cancer. Mice with defective DNA repair were generated by targeting the excision repair cross complementing gene (ERCC-1) in the embryonic stem cell line, HM-1. Homozygous ERCC-1 mutants were runted at birth and died before weaning with liver failure. Examination of organs revealed polyploidy in perinatal liver, progressing to severe aneuploidy by 3 weeks of age. Elevated p53 levels were detected in liver, brain and kidney, supporting the hypothesised role for p53 as a monitor of DNA damage.

引用

页码：217 / 224

页数：8

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