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Webbased Archive of RIVM Publications > Articles and other publications by RIVM employees > Substances > Tissue specific mutagenic and carcinogenic responses in NER defective mouse models.


Please use this identifier to cite or link to this item: http://hdl.handle.net/10029/8397
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Title: Tissue specific mutagenic and carcinogenic responses in NER defective mouse models.
Authors: Wijnhoven, Susan W P
Hoogervorst, Esther M
Waard, Harm de
Horst, Gijsbertus T J van der
Steeg, Harry van
Citation: Mutat. Res. 2007, 614(1-2):77-94
Issue Date: 3-Jan-2007
URI: http://hdl.handle.net/10029/8397
DOI: 10.1016/j.mrfmmm.2005.12.018
PubMed ID: 16769089
Abstract: Several mouse models with defects in genes encoding components of the nucleotide excision repair (NER) pathway have been developed. In NER two different sub-pathways are known, i.e. transcription-coupled repair (TC-NER) and global-genome repair (GG-NER). A defect in one particular NER protein can lead to a (partial) defect in GG-NER, TC-NER or both. GG-NER defects in mice predispose to cancer, both spontaneous as well as UV-induced. As such these models (Xpa, Xpc and Xpe) recapitulate the human xeroderma pigmentosum (XP) syndrome. Defects in TC-NER in humans are associated with Cockayne syndrome (CS), a disease not linked to tumor development. Mice with TC-NER defects (Csa and Csb) are - except for the skin - not susceptible to develop (carcinogen-induced) tumors. Some NER factors, i.e. XPB, XPD, XPF, XPG and ERCC1 have functions outside NER, like transcription initiation and inter-strand crosslink repair. Deficiencies in these processes in mice lead to very severe phenotypes, like trichothiodystrophy (TTD) or a combination of XP and CS. In most cases these animals have a (very) short life span, display segmental progeria, but do not develop tumors. Here we will overview the available NER-related mouse models and will discuss their phenotypes in terms of (chemical-induced) tissue-specific tumor development, mutagenesis and premature aging features.
Type: Article
Language: en
ISSN: 0027-5107
Appears in Collections:  Substances

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