Nucleic Acids Research, Vol 27, Issue 2 616-623, Copyright © 1999 by Oxford University Press
P Parniewski, A Bacolla, A Jaworski and RD Wells
The influence of nucleotide excision repair (NER), the principal in vivo
repair system for DNA damages, was investigated in Escherichia coli with
uvrA, uvrB and uvrAuvrB mutants with the triplet repeat sequences (TRS)
involved in myotonic dystrophy, the fragile X syndrome and Friedreich's
ataxia. (CTG*CAG)175was more stable when the (CTG) strand was transcribed
than when the (CAG) strand was transcribed in the alternate orientation. A
lack of the UvrA protein dramatically increases the instability of this TRS
in vivo as compared with the stability of the same sequence in uvrB mutant,
which produces an intact UvrA protein. We propose that transcription
transiently dissociates the triplet repeat complementary strands enabling
the non-transcribed strand to fold into a hairpin conformation which is
then sufficiently stable that replication bypasses the hairpin to give
large deletions. If the TRS was not transcribed, fewer deletions were
observed. Alternatively, in the uvrA-mutant, the hairpins existing on the
lagging strand will suffer bypass DNA synthesis to generate deleted
molecules. Hence, NER, functionally similar in both prokaryotes and
eukaryotes, is an important factor in the genetic instabilities of long
transcribed TRS implicated in human hereditary neuro-logical diseases.
ARTICLES
Nucleotide excision repair affects the stability of long transcribed (CTG*CAG) tracts in an orientation-dependent manner in Escherichia coli
Institute of Biosciences and Technology, Center for Genome Research, Department of Biochemistry and Biophysics, Texas A&M University, 2121 West Holcombe Boulevard, Houston, TX 77030-3303, USA.
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