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Cockayne Syndrome B protein antagonizes OGG1 in modulating CAG repeat length in vivo
| Citations: | 8 - 0 self |
BibTeX
@MISC{Kovtun_cockaynesyndrome,
author = {Irina V. Kovtun and Kurt O. Johnson and Cynthia T. Mcmurray},
title = {Cockayne Syndrome B protein antagonizes OGG1 in modulating CAG repeat length in vivo},
year = {}
}
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Abstract
Copyright: © Kovtun et al. This is an open‐access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited Δ Abstract: OGG1 and MSH2/MSH3 promote CAG repeat expansion at Huntingtons disease (HD) locus in vivo during removal of oxidized bases from DNA. CSB, a transcription‐coupled repair (TCR) protein, facilitates repair of some of the same oxidative lesions. In vitro, a knock down CSB results in a reduction of transcription‐induced deletions at CAG repeat tract. To test the role of CSB in vivo, we measured intergenerational and somatic expansion of CAG tracts in HD mice lacking CSB, OGG1, or both. We provide evidence that CSB protects CAG repeats from expansion by either active reduction of the tract length during parent‐child transmission, or by antagonizing the action of OGG1, which tends to promote expansion in somatic cells. These results raise a possibility that actions of transcription‐coupled and base excision repair pathways lead to different outcomes at CAG tracts in vivo.
Keyphrases
cag repeat length cockayne syndrome protein cag tract original author oxidative lesion cag repeat expansion creative common attribution license csb result somatic expansion base excision repair pathway tract length huntington disease unrestricted use cag repeat tract msh2 msh3 somatic cell oxidized base parent child transmission different outcome open access article active reduction cag repeat hd mouse
