Please use this identifier to cite or link to this item:
https://hdl.handle.net/10316/108913
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Lo Cicero, Alessandra | - |
dc.contributor.author | Jaskowiak, Anne-Laure | - |
dc.contributor.author | Egesipe, Anne-Laure | - |
dc.contributor.author | Tournois, Johana | - |
dc.contributor.author | Brinon, Benjamin | - |
dc.contributor.author | Pitrez, Patrícia R. | - |
dc.contributor.author | Ferreira, Lino | - |
dc.contributor.author | de Sandre-Giovannoli, Annachiara | - |
dc.contributor.author | Lévy, Nicolas | - |
dc.contributor.author | Nissan, Xavier | - |
dc.date.accessioned | 2023-09-25T08:46:15Z | - |
dc.date.available | 2023-09-25T08:46:15Z | - |
dc.date.issued | 2016-10-14 | - |
dc.identifier.issn | 2045-2322 | pt |
dc.identifier.uri | https://hdl.handle.net/10316/108913 | - |
dc.description.abstract | Hutchinson-Gilford progeria syndrome (HGPS) is a rare fatal genetic disorder that causes systemic accelerated aging in children. Thanks to the pluripotency and self-renewal properties of induced pluripotent stem cells (iPSC), HGPS iPSC-based modeling opens up the possibility of access to different relevant cell types for pharmacological approaches. In this study, 2800 small molecules were explored using high-throughput screening, looking for compounds that could potentially reduce the alkaline phosphatase activity of HGPS mesenchymal stem cells (MSCs) committed into osteogenic differentiation. Results revealed seven compounds that normalized the osteogenic differentiation process and, among these, all-trans retinoic acid and 13-cis-retinoic acid, that also decreased progerin expression. This study highlights the potential of high-throughput drug screening using HGPS iPS-derived cells, in order to find therapeutic compounds for HGPS and, potentially, for other aging-related disorders. | pt |
dc.language.iso | por | pt |
dc.publisher | Springer Nature | pt |
dc.relation | Institut National de la Santé et de la Recherche Médicale (INSERM), the National infrastructure Engineering for Pluripotent and differentiated Stem cells (INGESTEM), The region Ile-De-France (DIM Biothérapies), Evry Val d’Essonne University (UEVE), Association Française contre les Myopathies (AFM) and Genopole | pt |
dc.rights | openAccess | pt |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | pt |
dc.subject.mesh | Aging, Premature | pt |
dc.subject.mesh | Alkaline Phosphatase | pt |
dc.subject.mesh | Cell Differentiation | pt |
dc.subject.mesh | Child | pt |
dc.subject.mesh | Gene Expression Regulation | pt |
dc.subject.mesh | Guided Tissue Regeneration | pt |
dc.subject.mesh | High-Throughput Screening Assays | pt |
dc.subject.mesh | Humans | pt |
dc.subject.mesh | Induced Pluripotent Stem Cells | pt |
dc.subject.mesh | Isotretinoin | pt |
dc.subject.mesh | Lamin Type A | pt |
dc.subject.mesh | Mesenchymal Stem Cells | pt |
dc.subject.mesh | Osteogenesis | pt |
dc.subject.mesh | Progeria | pt |
dc.subject.mesh | Tretinoin | pt |
dc.title | A High Throughput Phenotypic Screening reveals compounds that counteract premature osteogenic differentiation of HGPS iPS-derived mesenchymal stem cells | pt |
dc.type | article | - |
degois.publication.firstPage | 34798 | pt |
degois.publication.issue | 1 | pt |
degois.publication.title | Scientific Reports | pt |
dc.peerreviewed | yes | pt |
dc.identifier.doi | 10.1038/srep34798 | pt |
degois.publication.volume | 6 | pt |
dc.date.embargo | 2016-10-14 | * |
uc.date.periodoEmbargo | 0 | pt |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
item.grantfulltext | open | - |
item.openairetype | article | - |
item.languageiso639-1 | pt | - |
item.fulltext | Com Texto completo | - |
item.cerifentitytype | Publications | - |
crisitem.author.orcid | 0000-0002-3116-0723 | - |
Appears in Collections: | I&D CNC - Artigos em Revistas Internacionais |
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This item is licensed under a Creative Commons License