Cited by 313Genetic Neurodegenerative DiseasesDNA and Nucleic Acid ChemistryGenetics and Neurodevelopmental Disorders

Sticky DNA

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Sakamoto, Naoaki, Chastain, Paul D., Parniewski, Pawel, Ohshima, Keiichi, Pandolfo, Massimo, Griffith, Jack D., Wells, Robert D.·1999·Molecular Cell

Abstract

A novel DNA structure, sticky DNA, is described for lengths of (GAA.TTC)n found in intron 1 of the frataxin gene of Friedreich's ataxia patients. Sticky DNA is formed by the association of two purine.purine.pyrimidine (R.R.Y) triplexes in negatively supercoiled plasmids at neutral pH. An excellent correlation was found between the lengths of (GAA.TTC) (> 59 repeats): first, in FRDA patients, second, required to inhibit transcription in vivo and in vitro, and third, required to adopt the sticky conformation. Fourth, (GAAGGA.TCCTTC)65, also found in intron 1, does not form sticky DNA, inhibit transcription, or associate with the disease. Hence, R.R.Y triplexes and/or sticky DNA may be involved in the etiology of FRDA.

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Crossref · 10.1016/s1097-2765(00)80474-8

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Authors
Sakamoto, N., Chastain, P. D., Parniewski, P., Ohshima, K., Pandolfo, M., Griffith, J. D., & Wells, R. D.
Affiliations
  • Texas Medical Center (US)
  • University of North Carolina at Chapel Hill (US)
  • Centre Hospitalier de l’Université de Montréal (CA)
Corresponding author
Wells, Robert D.
Venue
Molecular Cell
Publisher
Elsevier BV
Published
1999-04
Volume
3
Issue
4
Pages
465-475
ISSN
1097-2765 (ISSN-L)
OpenAlex
W146312310
Cited by
313
References
51
Citations by year
2026: 2 · 2025: 9 · 2024: 9 · 2023: 8 · 2022: 5 · 2021: 4 · 2020: 13 · 2019: 8 · 2018: 5 · 2017: 2 · 2016: 6 · 2015: 7 · 2014: 11 · 2013: 16 · 2012: 12
FWCI
6.63
Type
journal article
Language
en
Open access
Locations
  • Molecular Cell (publishedVersion)
  • PubMed (publishedVersion)
  • Dépôt institutionnel de l'Université libre de Bruxelles (Université Libre de Bruxelles) (submittedVersion)
Funders
  • Welch Foundation
  • National Institutes of Health (5p01gm052982-05, 7r01ns034192-06)
  • National Institute of General Medical Sciences (GM 52982)
  • National Institute of Neurological Disorders and Stroke (NS37544, NS34192)
Topics
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA and Nucleic Acid Chemistry
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Keywords
Friedreich's ataxia, frataxin gene, triplex DNA, intron 1, transcriptional inhibition, DNA supercoiling, etiology
MeSH terms
Frataxin, DNA, DNA, Superhelical, Electrophoresis, Agar Gel, Friedreich Ataxia, Humans, Microscopy, Electron, Models, Molecular, Nucleic Acid Conformation, Repetitive Sequences, Nucleic Acid, Transcription, Genetic, Phosphotransferases (Alcohol Group Acceptor), Iron-Binding Proteins

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