title: Organ and cell specificity of base excision repair mutants in mice.
abstract: Genetically modified mouse models are a powerful approach to study the relation of a single gene-deletion to processes such as mutagenesis and carcinogenesis. The generation of base excision repair (BER) deficient mouse models has resulted in a re-examination of the cellular defence mechanisms that exist to counteract DNA base damage. This review discusses novel insights into the relation between specific gene-deletions and the organ and cell specificity of visible and molecular phenotypes, including accumulation of base lesions in genomic DNA and carcinogenesis. Although promising models exist, there is still a need for new models. These models should comprise combined deficiencies of DNA glycosylases which initiate the BER pathway, to elaborate on the repair redundancy, as well as conditional models of the intermediate steps of BER.
authors.affiliation: Centre for Molecular Biology and Neuroscience, Institute of Medical Microbiology, Rikshospitalet-Radiumhospitalet HF, 0027 Oslo, Norway. elisabel@medisin.uio.no
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Larsen
authors.forename: Trine J
authors.initials: TJ
authors.lastname: Meza
authors.forename: Liv
authors.initials: L
authors.lastname: Kleppa
authors.forename: Arne
authors.initials: A
authors.lastname: Klungland
mesh_terms: D000818:Animals; D004249:DNA Damage; D045647:DNA Glycosylases / Q000235:genetics / Q000378:metabolism; D004260:DNA Repair / Q000235:genetics*; D045643:DNA Repair Enzymes / Q000235:genetics / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D008957:Models, Genetic; D009154:Mutation; D009928:Organ Specificity
chemical_list: D045647:DNA Glycosylases; D045643:DNA Repair Enzymes