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

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 telome...

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Autores principales: 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
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Lenguaje:EN
Publicado: Nature Portfolio 2019
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Acceso en línea:https://doaj.org/article/d5eb9edbff7e43f5b790e3b311b3399f
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spelling oai:doaj.org-article:d5eb9edbff7e43f5b790e3b311b3399f2021-12-02T16:50:59ZInhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome10.1038/s41467-019-13018-32041-1723https://doaj.org/article/d5eb9edbff7e43f5b790e3b311b3399f2019-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-019-13018-3https://doaj.org/toc/2041-1723Hutchinson–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.Julio AguadoAgustin Sola-CarvajalValeria CancilaGwladys RevêchonPeh Fern OngCorey Winston Jones-WeinertEmelie Wallén ArztGiovanna LattanziOliver DreesenClaudio TripodoFrancesca RossielloMaria ErikssonFabrizio d’Adda di FagagnaNature PortfolioarticleScienceQENNature Communications, Vol 10, Iss 1, Pp 1-11 (2019)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
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
Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
description 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.
format article
author 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
author_facet 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
author_sort Julio Aguado
title Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
title_short Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
title_full Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
title_fullStr Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
title_full_unstemmed Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson–Gilford Progeria Syndrome
title_sort inhibition of dna damage response at telomeres improves the detrimental phenotypes of hutchinson–gilford progeria syndrome
publisher Nature Portfolio
publishDate 2019
url https://doaj.org/article/d5eb9edbff7e43f5b790e3b311b3399f
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