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dc.contributor.authorFlores, Juana M
dc.contributor.authorBlasco, MA 
dc.contributor.authorMulero, Francisca 
dc.contributor.authorMartinez Rodriguez, Paula 
dc.date.accessioned2020-06-09T16:34:38Z
dc.date.available2020-06-09T16:34:38Z
dc.date.issued2015-07-14
dc.identifier.citationCell Rep. 2015;12(2):286-99.es_ES
dc.identifier.urihttp://hdl.handle.net/20.500.12105/10312
dc.description.abstractIdiopathic pulmonary fibrosis (IPF) is a degenerative disease of the lungs with an average survival post-diagnosis of 2-3 years. New therapeutic targets and treatments are necessary. Mutations in components of the telomere-maintenance enzyme telomerase or in proteins important for telomere protection are found in both familial and sporadic IPF cases. However, the lack of mouse models that faithfully recapitulate the human disease has hampered new advances. Here, we generate two independent mouse models that develop IPF owing to either critically short telomeres (telomerase-deficient mice) or severe telomere dysfunction in the absence of telomere shortening (mice with Trf1 deletion in type II alveolar cells). We show that both mouse models develop pulmonary fibrosis through induction of telomere damage, thus providing proof of principle of the causal role of DNA damage stemming from dysfunctional telomeres in IPF development and identifying telomeres as promising targets for new treatments.es_ES
dc.description.sponsorshipResearch in the Blasco lab is funded by the Spanish Ministry of Economy and Competitiveness Projects SAF2008-05384 and CSD2007-00017, the European Union FP7 Projects 2007-A-201630 (GENICA) and 2007-A-200950 (TELOMARKER), the European Research Council (ERC) Project TEL STEM CELL (GA#232854), the Korber Foundation, the AXA Research Fund, Fundacion Botin, and Fundacion Lilly (Spain). F.B. is ICREA Academia, Generalitat de Catalunya, Spain.es_ES
dc.language.isoenges_ES
dc.publisherCell Presses_ES
dc.relation.isversionofPublisher's versiones_ES
dc.rights.urihttp://creativecommons.org/licenses/by-nc-sa/4.0/*
dc.subject.meshAnimals es_ES
dc.subject.meshCyclin-Dependent Kinase Inhibitor p21 es_ES
dc.subject.meshDNA Damage es_ES
dc.subject.meshDNA Repair es_ES
dc.subject.meshDisease Models, Animales_ES
dc.subject.meshFemale es_ES
dc.subject.meshIdiopathic Pulmonary Fibrosis es_ES
dc.subject.meshLung es_ES
dc.subject.meshMale es_ES
dc.subject.meshMice es_ES
dc.subject.meshMice, Inbred C57BL es_ES
dc.subject.meshMice, Knockout es_ES
dc.subject.meshRadiography es_ES
dc.subject.meshTamoxifen es_ES
dc.subject.meshTelomerase es_ES
dc.subject.meshTelomere es_ES
dc.subject.meshTelomere Shortening es_ES
dc.subject.meshTelomeric Repeat Binding Protein 1 es_ES
dc.subject.meshFailurees_ES
dc.titleMice with Pulmonary Fibrosis Driven by Telomere Dysfunction.es_ES
dc.typeArtículoes_ES
dc.rights.licenseAtribución-NoComercial-CompartirIgual 4.0 Internacional*
dc.identifier.pubmedID26146081es_ES
dc.format.volume12es_ES
dc.format.number2es_ES
dc.format.page286-99es_ES
dc.identifier.doi10.1016/j.celrep.2015.06.028es_ES
dc.contributor.funderMinisterio de Economía y Competitividad (España)es_ES
dc.contributor.funderEuropean Union (EU)es_ES
dc.contributor.funderEuropean Research Council (ERC)es_ES
dc.contributor.funderKorber Foundationes_ES
dc.contributor.funderAXA Research Fundes_ES
dc.contributor.funderFundacion Botínes_ES
dc.contributor.funderFundacion Lilly (Spain)es_ES
dc.description.peerreviewedes_ES
dc.identifier.e-issn2211-1247es_ES
dc.relation.publisherversionhttps://doi.org/10.1016/j.celrep.2015.06.028es_ES
dc.identifier.journalCell reportses_ES
dc.repisalud.institucionCNIOes_ES
dc.repisalud.orgCNIOCNIO::Grupos de investigación::Grupo de Telómeros y Telomerasaes_ES
dc.relation.projectIDinfo:eu_repo/grantAgreement/EC/FP7/201630es_ES
dc.relation.projectIDinfo:eu_repo/grantAgreement/ES/SAF2008-0538es_ES
dc.rights.accessRightsinfo:eu-repo/semantics/openAccesses_ES


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Atribución-NoComercial-CompartirIgual 4.0 Internacional
This item is licensed under a: Atribución-NoComercial-CompartirIgual 4.0 Internacional