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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
1997-6-6
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pubmed:abstractText |
A mouse model for the nucleotide excision repair disorder Cockayne syndrome (CS) was generated by mimicking a truncation in the CSB(ERCC6) gene of a CS-B patient. CSB-deficient mice exhibit all of the CS repair characteristics: ultraviolet (UV) sensitivity, inactivation of transcription-coupled repair, unaffected global genome repair, and inability to resume RNA synthesis after UV exposure. Other CS features thought to involve the functioning of basal transcription/repair factor TFIIH, such as growth failure and neurologic dysfunction, are present in mild form. In contrast to the human syndrome, CSB-deficient mice show increased susceptibility to skin cancer. Our results demonstrate that transcription-coupled repair of UV-induced cyclobutane pyrimidine dimers contributes to the prevention of carcinogenesis in mice. Further, they suggest that the lack of cancer predisposition in CS patients is attributable to a global genome repair process that in humans is more effective than in rodents.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/DNA Helicases,
http://linkedlifedata.com/resource/pubmed/chemical/DNA Repair Enzymes,
http://linkedlifedata.com/resource/pubmed/chemical/ERCC6 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Ercc6 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Repressor Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Transcription Factors,
http://linkedlifedata.com/resource/pubmed/chemical/Viral Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Viral Regulatory and Accessory...,
http://linkedlifedata.com/resource/pubmed/chemical/phage repressor proteins
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pubmed:status |
MEDLINE
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pubmed:month |
May
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pubmed:issn |
0092-8674
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pubmed:author |
pubmed-author:AONOKK,
pubmed-author:BeemsR BRB,
pubmed-author:BootsmaDD,
pubmed-author:HoeijmakersJ HJH,
pubmed-author:MorreauHH,
pubmed-author:WeedaGG,
pubmed-author:de GruijlF RFR,
pubmed-author:de WitJJ,
pubmed-author:van GoolA JAJ,
pubmed-author:van KreijlC FCF,
pubmed-author:van SteegHH,
pubmed-author:van der HorstG TGT
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pubmed:issnType |
Print
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pubmed:day |
2
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pubmed:volume |
89
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
425-35
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pubmed:dateRevised |
2007-11-15
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pubmed:meshHeading |
pubmed-meshheading:9150142-Alleles,
pubmed-meshheading:9150142-Amino Acid Sequence,
pubmed-meshheading:9150142-Animals,
pubmed-meshheading:9150142-Cockayne Syndrome,
pubmed-meshheading:9150142-DNA Helicases,
pubmed-meshheading:9150142-DNA Repair,
pubmed-meshheading:9150142-DNA Repair Enzymes,
pubmed-meshheading:9150142-Disease Models, Animal,
pubmed-meshheading:9150142-Female,
pubmed-meshheading:9150142-Humans,
pubmed-meshheading:9150142-Male,
pubmed-meshheading:9150142-Mice,
pubmed-meshheading:9150142-Mice, Inbred C57BL,
pubmed-meshheading:9150142-Mice, Mutant Strains,
pubmed-meshheading:9150142-Mutagenesis,
pubmed-meshheading:9150142-Photosensitivity Disorders,
pubmed-meshheading:9150142-Repressor Proteins,
pubmed-meshheading:9150142-Skin Neoplasms,
pubmed-meshheading:9150142-Transcription, Genetic,
pubmed-meshheading:9150142-Transcription Factors,
pubmed-meshheading:9150142-Ultraviolet Rays,
pubmed-meshheading:9150142-Viral Proteins,
pubmed-meshheading:9150142-Viral Regulatory and Accessory Proteins
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pubmed:year |
1997
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pubmed:articleTitle |
Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predisposition.
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pubmed:affiliation |
Medical Genetics Center, Department of Cell Biology and Genetics, Erasmus University Rotterdam, The Netherlands.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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