DOI: 10.1126/science.275.5302.990. Defective Transcription-Coupled Repair of Oxidative Base Damage in Cockayne Syndrome Patients from XP Group G

Defective Transcription-Coupled Repair of Oxidative Base Damage in Cockayne Syndrome Patients from XP Group G

10.1126/science.275.5302.990

Crossref journal-article

American Association for the Advancement of Science (AAAS)

Science (221)

Abstract

In normal human cells, damage due to ultraviolet light is preferentially removed from active genes by nucleotide excision repair (NER) in a transcription-coupled repair (TCR) process that requires the gene products defective in Cockayne syndrome (CS). Oxidative damage, including thymine glycols, is shown to be removed by TCR in cells from normal individuals and from xeroderma pigmentosum (XP)-A, XP-F, and XP-G patients who have NER defects but not from XP-G patients who have severe CS. Thus, TCR of oxidative damage requires an XPG function distinct from its NER endonuclease activity. These results raise the possibility that defective TCR of oxidative damage contributes to the developmental defects associated with CS.

Bibliography

Cooper, P. K., Nouspikel, T., Clarkson, S. G., & Leadon, S. A. (1997). Defective Transcription-Coupled Repair of Oxidative Base Damage in Cockayne Syndrome Patients from XP Group G. Science, 275(5302), 990â993.

Authors 4

Priscilla K. Cooper (first)
Thierry Nouspikel (additional)
Stuart G. Clarkson (additional)
Steven A. Leadon (additional)

References 47 Referenced 239

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We thank C. Arlett (XP125LO) and W. Vermeulen and N. G. J. Jaspers (three XP-G/CS) for cell strains and E. E. Kwoh C. Ng and A. Avrutskaya for technical assistance. Supported by grants from NIH (CA63503) and DOE (through contract DE-AC03-76SF00098) to P.K.C. from NIH (CA40453) to S.A.L. and from the Swiss National Science Foundation (31-36482.92) to S.G.C.

Dates

Type	When
Created	23 years, 1 month ago (July 27, 2002, 5:45 a.m.)
Deposited	1 year, 7 months ago (Jan. 12, 2024, 11:06 p.m.)
Indexed	4 months, 3 weeks ago (April 3, 2025, 8:31 p.m.)
Issued	28 years, 6 months ago (Feb. 14, 1997)
Published	28 years, 6 months ago (Feb. 14, 1997)
Published Print	28 years, 6 months ago (Feb. 14, 1997)

Funders 0

None

BibTeX

@article{Cooper_1997, title={Defective Transcription-Coupled Repair of Oxidative Base Damage in Cockayne Syndrome Patients from XP Group G}, volume={275}, ISSN={1095-9203}, url={http://dx.doi.org/10.1126/science.275.5302.990}, DOI={10.1126/science.275.5302.990}, number={5302}, journal={Science}, publisher={American Association for the Advancement of Science (AAAS)}, author={Cooper, Priscilla K. and Nouspikel, Thierry and Clarkson, Stuart G. and Leadon, Steven A.}, year={1997}, month=feb, pages={990–993} }

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