Tissue specific mutagenic and carcinogenic responses in NER defective mouse models.

2.50
Hdl Handle:
http://hdl.handle.net/10029/8397
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
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.
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
Type:
Article
Language:
en
ISSN:
0027-5107
Appears in Collections:
Substances

Full metadata record

DC FieldValue Language
dc.contributor.authorWijnhoven, Susan W P-
dc.contributor.authorHoogervorst, Esther M-
dc.contributor.authorWaard, Harm de-
dc.contributor.authorHorst, Gijsbertus T J van der-
dc.contributor.authorSteeg, Harry van-
dc.date.accessioned2007-02-14T15:16:24Z-
dc.date.available2007-02-14T15:16:24Z-
dc.date.issued2007-01-03-
dc.identifier.citationMutat. Res. 2007, 614(1-2):77-94en
dc.identifier.issn0027-5107-
dc.identifier.pmid16769089-
dc.identifier.doi10.1016/j.mrfmmm.2005.12.018-
dc.identifier.urihttp://hdl.handle.net/10029/8397-
dc.description.abstractSeveral 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.en
dc.format.extent522573 bytes-
dc.format.mimetypeapplication/pdf-
dc.language.isoenen
dc.titleTissue specific mutagenic and carcinogenic responses in NER defective mouse models.en
dc.typeArticleen
dc.format.digYES-

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