DOI: 10.1073/pnas.94.17.9463. <i>In vitro</i> repair of oxidative DNA damage by human nucleotide excision repair system: Possible explanation for neurodegeneration in Xeroderma pigmentosum patients

In vitro repair of oxidative DNA damage by human nucleotide excision repair system: Possible explanation for neurodegeneration in Xeroderma pigmentosum patients

10.1073/pnas.94.17.9463

Crossref journal-article

Proceedings of the National Academy of Sciences

Proceedings of the National Academy of Sciences (341)

Abstract

Xeroderma pigmentosum (XP) patients fail to remove pyrimidine dimers caused by sunlight and, as a consequence, develop multiple cancers in areas exposed to light. The second most common sign, present in 20–30% of XP patients, is a set of neurological abnormalities caused by neuronal death in the central and peripheral nervous systems. Neural tissue is shielded from sunlight-induced DNA damage, so the cause of neurodegeneration in XP patients remains unexplained. In this study, we show that two major oxidative DNA lesions, 8-oxoguanine and thymine glycol, are excised from DNA in vitro by the same enzyme system responsible for removing pyrimidine dimers and other bulky DNA adducts. Our results suggest that XP neurological disease may be caused by defective repair of lesions that are produced in nerve cells by reactive oxygen species generated as by-products of an active oxidative metabolism.

Bibliography

Reardon, J. T., Bessho, T., Kung, H. C., Bolton, P. H., & Sancar, A. (1997). In vitro repair of oxidative DNA damage by human nucleotide excision repair system: Possible explanation for neurodegeneration in Xeroderma pigmentosumâpatients. Proceedings of the National Academy of Sciences, 94(17), 9463â9468.

Authors 5

Joyce T. Reardon (first)
Tadayoshi Bessho (additional)
Hsiang Chuan Kung (additional)
Philip H. Bolton (additional)
Aziz Sancar (additional)

References 54 Referenced 282

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Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 10:40 a.m.)
Deposited	3 years, 4 months ago (April 13, 2022, 2:27 p.m.)
Indexed	1 week, 6 days ago (Aug. 21, 2025, 12:47 p.m.)
Issued	28 years ago (Aug. 19, 1997)
Published	28 years ago (Aug. 19, 1997)
Published Online	28 years ago (Aug. 19, 1997)
Published Print	28 years ago (Aug. 19, 1997)

Funders 0

None

BibTeX

@article{Reardon_1997, title={In vitro repair of oxidative DNA damage by human nucleotide excision repair system: Possible explanation for neurodegeneration in Xeroderma pigmentosum patients}, volume={94}, ISSN={1091-6490}, url={http://dx.doi.org/10.1073/pnas.94.17.9463}, DOI={10.1073/pnas.94.17.9463}, number={17}, journal={Proceedings of the National Academy of Sciences}, publisher={Proceedings of the National Academy of Sciences}, author={Reardon, Joyce T. and Bessho, Tadayoshi and Kung, Hsiang Chuan and Bolton, Philip H. and Sancar, Aziz}, year={1997}, month=aug, pages={9463–9468} }

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