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Dual functions of DNA repair genes: molecular, cellular, and clinical implications
Author(s) -
Lehmann A.R.
Publication year - 1998
Publication title -
bioessays
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.175
H-Index - 184
eISSN - 1521-1878
pISSN - 0265-9247
DOI - 10.1002/(sici)1521-1878(199802)20:2<146::aid-bies7>3.0.co;2-r
Subject(s) - transcription factor ii h , xeroderma pigmentosum , nucleotide excision repair , dna repair , cockayne syndrome , biology , genetics , gene , transcription factor , transcription (linguistics) , dna , computational biology , microbiology and biotechnology , linguistics , philosophy
The complex series of DNA repair pathways that are used to repair damage to cellular DNA employ many different proteins. A substantial number of these have second functions. Defects in these multifunctional proteins in man can lead to widely differing clinical phenotypes depending on which of the functions is affected. This is illustrated most clearly in the transcription factor TFIIH, which is involved in both basal transcription and nucleotide excision repair. Different mutations in genes encoding TFIIH subunits can result in the highly cancer‐prone repair disorder xeroderma pigmentosum, or the noncancer‐prone multisystem disorder trichothiodystrophy, the features of which are probably a consequence of abnormalities in transcription. The involvement of repair proteins in other processes also poses interesting evolutionary questions. BioEssays 20:146–155, 1998. © 1998 John Wiley & Sons, Inc.