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Cellular and Molecular Life Sciences: CMLS logoLink to Cellular and Molecular Life Sciences: CMLS
. 2009 Jan 21;66(6):994–1009. doi: 10.1007/s00018-009-8737-y

DNA Repair in Mammalian Cells

Nucleotide excision repair: variations on versatility

T Nouspikel 1,
PMCID: PMC11131503  PMID: 19153657

Abstract.

Nucleotide excision repair (NER) is one of the most versatile DNA repair systems. It can be subdivided into several, differentially regulated, subpathways: global genome repair (GGR), transcription-coupled repair (TCR), and transcription domain-associated repair (DAR). This review begins with a brief overview of the numerous types of DNA lesions handled by NER, and proceeds to describe in detail the molecular mechanisms of NER. It then addresses heterogeneities in NER activity in physiological situations (e. g. in differentiated cells) and explores the underlying regulatory mechanism. It then reviews several inherited diseases associated with NER deficiencies: xeroderma pigmentosum, Cockayne syndrome, trichothiodystrophy, UV-sensitive syndrome. It concludes by discussing several currently unresolved issues, relating either to the cause of the above diseases or to the mechanistic details of the various NER subpathways and of their regulation. (Part of a Multi-author Review)

Keywords. DNA repair, nucleotide excision repair, transcription-coupled repair, domain-associated repair, xeroderma pigmentosum, Cockayne syndrome, trichothiodystrophy, UV-sensitive syndrome, cellular differentiation


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