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- Aguado, J, et al.
(author)
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Inhibition of DNA damage response at telomeres improves the detrimental phenotypes of Hutchinson-Gilford Progeria Syndrome
- 2019
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In: Nature communications. - : Springer Science and Business Media LLC. - 2041-1723. ; 10:1, s. 4990-
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Journal article (peer-reviewed)abstract
- Hutchinson–Gilford progeria syndrome (HGPS) is a genetic disorder characterized by premature aging features. Cells from HGPS patients express progerin, a truncated form of Lamin A, which perturbs cellular homeostasis leading to nuclear shape alterations, genome instability, heterochromatin loss, telomere dysfunction and premature entry into cellular senescence. Recently, we reported that telomere dysfunction induces the transcription of telomeric non-coding RNAs (tncRNAs) which control the DNA damage response (DDR) at dysfunctional telomeres. Here we show that progerin-induced telomere dysfunction induces the transcription of tncRNAs. Their functional inhibition by sequence-specific telomeric antisense oligonucleotides (tASOs) prevents full DDR activation and premature cellular senescence in various HGPS cell systems, including HGPS patient fibroblasts. We also show in vivo that tASO treatment significantly enhances skin homeostasis and lifespan in a transgenic HGPS mouse model. In summary, our results demonstrate an important role for telomeric DDR activation in HGPS progeroid detrimental phenotypes in vitro and in vivo.
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- Cancila, D., et al.
(author)
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Experiences and reflections on three years of CPS Summer schools within EIT digital
- 2016
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In: 2016 Workshop on Embedded and Cyber-Physical Systems Education, WESE 2016 - Organized as a Part of Embedded Systems Week, Proceedings. - New York, NY, USA : ACM Digital Library. - 9781450346573
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Conference paper (peer-reviewed)abstract
- This article provides an overview of current European Commission effort in term of educational innovation to reduce the gap between research and industry which still is a barrier to the economic development. Entrepreneurial innovation & education driving Europe's digital transformation (EIT Digital for short) is an European-based initiative fostering I&E (innovation and entrepreneurship) by integrating education, research and business at different educational levels. For instance in EIT master programmes, students work together with industries and academics to have a faster go-to-market of research results. Summer schools are part of the master programs; three of them have been organised related to CPS (cyber-physical systems), critical infrastructure and, more recently, Industry 4.0. Past and present events are discussed and the experience from these events is reported. It is further analysed how the general setup of the summer school program is affecting the educational aspects and achievement of the intended learning outcomes.
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