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dc.contributor.authorVaretti, Gianluca
dc.contributor.authorGómez-López, Gonzalo 
dc.contributor.authorMartinez, Dolores
dc.date.accessioned2020-06-08T16:34:16Z
dc.date.available2020-06-08T16:34:16Z
dc.date.issued2015-10-27
dc.identifier.citationCell Presses_ES
dc.identifier.urihttp://hdl.handle.net/20.500.12105/10290
dc.description.abstractDiamond-Blackfan anemia (DBA) is characterized by anemia and cancer susceptibility and is caused by mutations in ribosomal genes, including RPL11. Here, we report that Rpl11-heterozygous mouse embryos are not viable and that Rpl11 homozygous deletion in adult mice results in death within a few weeks, accompanied by bone marrow aplasia and intestinal atrophy. Importantly, Rpl11 heterozygous deletion in adult mice results in anemia associated with decreased erythroid progenitors and defective erythroid maturation. These defects are also present in mice transplanted with inducible heterozygous Rpl11 bone marrow and, therefore, are intrinsic to the hematopoietic system. Additionally, heterozygous Rpl11 mice present increased susceptibility to radiation-induced lymphomagenesis. In this regard, total or partial deletion of Rpl11 compromises p53 activation upon ribosomal stress or DNA damage in fibroblasts. Moreover, fibroblasts and hematopoietic tissues from heterozygous Rpl11 mice present higher basal cMYC levels. We conclude that Rpl11-deficient mice recapitulate DBA disorder, including cancer predisposition.es_ES
dc.description.sponsorshipWe thank M. Foronda, M. Abad, C. Pantoja, P.J. Fernandez-Marcos, A. de Martino, G. Iglesias, O. Dominguez, and S. Ortega for valuable contributions. We are also thankful to the Flow Cytometry, Genomics, Transgenics, and Histopathology Units of the CNIO. L.M.-P. was recipient of a predoctoral fellowship from the Spanish Ministry of Education. Work in the laboratory of M. S. is funded by the CNIO and by grants from the Spanish Ministry of Economy (SAF), the European Research Council (ERC Advanced Grant), the Regional Government of Madrid, the Botin Foundation and Banco Santander (Santander Universities Global Division), the Ramon Areces Foundation, and the AXA Foundation.es_ES
dc.language.isoenges_ES
dc.type.hasVersionVoRes_ES
dc.rights.urihttp://creativecommons.org/licenses/by-nc-sa/4.0/*
dc.subjectEXPRESSIONes_ES
dc.subjectMUTATIONSes_ES
dc.subjectRIBOSOMAL-PROTEIN L11es_ES
dc.subjectC-MYCes_ES
dc.subjectP53 ACTIVATIONes_ES
dc.subjectERYTHROID-DIFFERENTIATIONes_ES
dc.subject.meshAnemia, Diamond-Blackfan es_ES
dc.subject.meshAnimals es_ES
dc.subject.meshCell Proliferation es_ES
dc.subject.meshCells, Cultured es_ES
dc.subject.meshErythroid Cells es_ES
dc.subject.meshLymphoma es_ES
dc.subject.meshMice es_ES
dc.subject.meshMice, Knockout es_ES
dc.subject.meshRibosomal Proteins es_ES
dc.subject.meshTumor Suppressor Protein p53 es_ES
dc.titlePartial Loss of Rpl11 in Adult Mice Recapitulates Diamond-Blackfan Anemia and Promotes Lymphomagenesis.es_ES
dc.typejournal articlees_ES
dc.rights.licenseAtribución-NoComercial-CompartirIgual 4.0 Internacional*
dc.identifier.pubmedID26489471es_ES
dc.format.volume13es_ES
dc.format.number4es_ES
dc.format.page712-722es_ES
dc.identifier.doi10.1016/j.celrep.2015.09.038es_ES
dc.contributor.funderMinisterio de Economía y Competitividad (España) 
dc.contributor.funderUnión Europea. Comisión Europea. European Research Council (ERC) 
dc.contributor.funderComunidad de Madrid (España) 
dc.contributor.funderBotín Foundation 
dc.contributor.funderFundación Ramón Areces 
dc.contributor.funderFundación AXA 
dc.contributor.funderCentro Nacional de Investigaciones Oncológicas Carlos III (España) 
dc.description.peerreviewedes_ES
dc.identifier.e-issn2211-1247es_ES
dc.relation.publisherversionhttps://doi.org/10.1016/j.celrep.2015.09.038es_ES
dc.identifier.journalCell reportses_ES
dc.repisalud.institucionCNIOes_ES
dc.repisalud.orgCNIOCNIO::Grupos de investigación::Antiguos CNIOes_ES
dc.rights.accessRightsopen accesses_ES


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