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Pathogenic CANVAS-causing but not nonpathogenic RFC1 DNA/RNA repeat motifs form quadruplex or triplex structures.

Mohammad Hossein Abdi1, Bita Zamiri1, Gholamreza Pazuki1

  • 1Department of Chemical Engineering, Amirkabir University of Technology (Tehran Polytechnic), Tehran, Iran.

The Journal of Biological Chemistry
|September 3, 2023
PubMed
Summary

Pathogenic repeat expansions in RFC1 cause CANVAS by forming unusual G-quadruplex and triple-stranded nucleic acid structures. These structures, unlike nonpathogenic repeats, may drive toxic-DNA and toxic-RNA pathogenesis.

Keywords:
CANVASDNAG-quadruplexRFC1RNAataxiaspectroscopytriplex DNA

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Area of Science:

  • Genetics
  • Molecular Biology
  • Neuroscience

Background:

  • Biallelic expansions in RFC1 are linked to CANVAS, but the specific pathogenic mechanisms and repeat motifs remain unclear.
  • While various repeat motifs can expand, only certain ones trigger the specific symptoms of CANVAS.
  • The common pathogenic repeat is (AAGGG)n•(CCCTT)n, contrasting with the nonpathogenic (AAAAG)n•(CTTTT)n.

Purpose of the Study:

  • To elucidate the structural basis for pathogenicity in RFC1 repeat expansions.
  • To understand why specific repeat motifs cause CANVAS while others do not.
  • To explore potential therapeutic targets based on the structural properties of pathogenic repeats.

Main Methods:

  • Investigated nucleic acid structural formation (G-quadruplexes, triplexes) by pathogenic and nonpathogenic RFC1 repeat motifs in DNA and RNA.
  • Utilized potassium solutions to assess G-quadruplex formation.
  • Examined the binding of the ligand TMPyP4 to pathogenic quadruplex structures.

Main Results:

  • Pathogenic repeat motifs (AAGGG)n and (AAAAG)n formed G-quadruplexes and triple-stranded structures in DNA and RNA.
  • Nonpathogenic repeats failed to form these unusual nucleic acid structures.
  • The G- and C-rich nature of pathogenic repeats facilitates G-quadruplex and triplex formation through Hoogsteen base pairing and stabilized interactions.
  • TMPyP4 ligand specifically bound to the pathogenic quadruplexes.

Conclusions:

  • Unusual nucleic acid structures, including G-quadruplexes and triplexes, are formed by pathogenic RFC1 repeat expansions, supporting toxic-DNA and toxic-RNA pathogenesis.
  • The sequence-specific structural properties of repeat motifs determine their pathogenicity in CANVAS.
  • These findings offer insights into disease specificity and identify potential therapeutic targets for CANVAS.