Skip to main content

NOTICE: Due to a lapse in annual appropriations, most of this website is not being updated. Learn more.

Form submissions will still be accepted but will not receive responses at this time. Sections of this site for programs using non-appropriated funds (such as NVLAP) or those that are excepted from the shutdown (such as CHIPS and NVD) will continue to be updated.

U.S. flag

An official website of the United States government

Official websites use .gov
A .gov website belongs to an official government organization in the United States.

Secure .gov websites use HTTPS
A lock ( ) or https:// means you’ve safely connected to the .gov website. Share sensitive information only on official, secure websites.

The Cockayne Syndrome Group B Gene Product is Involved in Cellular Repair of 8-Hydroxyadenine in DNA

Published

Author(s)

J Tuo, Pawel Jaruga, H Rodriguez, Miral M. Dizdar, V. Bohr

Abstract

Cockayne syndrome (CS) is a human disease characterized by sensitivity to sunlight, severe neurological abnormalities and accelerated aging. CS has two complementation groups, CS-A and CS-B. The CSB gene encodes the CSB protein with 1493 amino acids. We previously reported that the CSB protein is involved in cellular repair of 8-hydroxyguanine, an abundant lesion in oxidatively damaged DNA, and that the putative helicase motif V/VI of the CSB may play a role in this process. The present study investigated the role of the CSB protein in cellular repair of 8-hydroxyadenine, another abundant lesion in oxidatively damaged DNA. Extracts of CS-B null cells and mutant cells with site-directed mutation in the motif VI of the putative helicase domain incised 8-hydroxyadenine in vitro less efficiently than wild type cells. Furthermore, CS-B null and motif VI mutant cells accumulated more 8-hydroxyadenine in their genomic DNA than wild type cells after exposure to γ-radiation at doses of 2 or 5 Gy. These results suggest that the CSB protein contributes to cellular repair of 8-OH-Ade, and that the motif VI of the putative helicase domain of CSB is required by this activity.
Citation
Journal of Biological Chemistry
Volume
277
Issue
34

Keywords

8-hydroxyadenine, cockayne syndrome, DNA repair, liquid chromatography/mass spectrometry, oxidative DNA damage

Citation

Tuo, J. , Jaruga, P. , Rodriguez, H. , Dizdar, M. and Bohr, V. (2002), The Cockayne Syndrome Group B Gene Product is Involved in Cellular Repair of 8-Hydroxyadenine in DNA, Journal of Biological Chemistry (Accessed October 14, 2025)

Issues

If you have any questions about this publication or are having problems accessing it, please contact [email protected].

Created August 22, 2002, Updated October 12, 2021
Was this page helpful?