Nature Communications (Nov 2019)

Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome

  • Julio Aguado,
  • Agustin Sola-Carvajal,
  • Valeria Cancila,
  • Gwladys Revêchon,
  • Peh Fern Ong,
  • Corey Winston Jones-Weinert,
  • Emelie Wallén Arzt,
  • Giovanna Lattanzi,
  • Oliver Dreesen,
  • Claudio Tripodo,
  • Francesca Rossiello,
  • Maria Eriksson,
  • Fabrizio d’Adda di Fagagna

DOI
https://doi.org/10.1038/s41467-019-13018-3
Journal volume & issue
Vol. 10, no. 1
pp. 1 – 11

Abstract

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Hutchinson–Gilford progeria syndrome causes premature aging. Here the authors show that activation of the DNA damage response at dysfunctional telomeres and transcription of telomeric non-coding RNAs contributes to the pathogenesis, which can be ameliorated by treatment with sequence-specific telomeric antisense oligonucleotides.