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Items: 32


Development of a CRISPR/Cas9-based therapy for Hutchinson-Gilford progeria syndrome.

Santiago-Fernández O, Osorio FG, Quesada V, Rodríguez F, Basso S, Maeso D, Rolas L, Barkaway A, Nourshargh S, Folgueras AR, Freije JMP, López-Otín C.

Nat Med. 2019 Mar;25(3):423-426. doi: 10.1038/s41591-018-0338-6. Epub 2019 Feb 18.


Retraction Note: NF-κB activation impairs somatic cell reprogramming in ageing.

Soria-Valles C, Osorio FG, Gutiérrez-Fernández A, De Los Angeles A, Bueno C, Menéndez P, Martín-Subero JI, Daley GQ, Freije JMP, López-Otín C.

Nat Cell Biol. 2019 Mar;21(3):410. doi: 10.1038/s41556-018-0259-0.


Somatic Mutations Reveal Lineage Relationships and Age-Related Mutagenesis in Human Hematopoiesis.

Osorio FG, Rosendahl Huber A, Oka R, Verheul M, Patel SH, Hasaart K, de la Fonteijne L, Varela I, Camargo FD, van Boxtel R.

Cell Rep. 2018 Nov 27;25(9):2308-2316.e4. doi: 10.1016/j.celrep.2018.11.014.


Changes at the nuclear lamina alter binding of pioneer factor Foxa2 in aged liver.

Whitton H, Singh LN, Patrick MA, Price AJ, Osorio FG, López-Otín C, Bochkis IM.

Aging Cell. 2018 Jun;17(3):e12742. doi: 10.1111/acel.12742. Epub 2018 Feb 27.


Cardiac electrical defects in progeroid mice and Hutchinson-Gilford progeria syndrome patients with nuclear lamina alterations.

Rivera-Torres J, Calvo CJ, Llach A, Guzmán-Martínez G, Caballero R, González-Gómez C, Jiménez-Borreguero LJ, Guadix JA, Osorio FG, López-Otín C, Herraiz-Martínez A, Cabello N, Vallmitjana A, Benítez R, Gordon LB, Jalife J, Pérez-Pomares JM, Tamargo J, Delpón E, Hove-Madsen L, Filgueiras-Rama D, Andrés V.

Proc Natl Acad Sci U S A. 2016 Nov 15;113(46):E7250-E7259. Epub 2016 Oct 31.


Immune and inflammatory responses to DNA damage in cancer and aging.

Soria-Valles C, López-Soto A, Osorio FG, López-Otín C.

Mech Ageing Dev. 2017 Jul;165(Pt A):10-16. doi: 10.1016/j.mad.2016.10.004. Epub 2016 Oct 5. Review.


NF-κB signaling as a driver of ageing.

Osorio FG, Soria-Valles C, Santiago-Fernández O, Freije JM, López-Otín C.

Int Rev Cell Mol Biol. 2016;326:133-74. doi: 10.1016/bs.ircmb.2016.04.003. Epub 2016 May 30. Review.


MMP-25 Metalloprotease Regulates Innate Immune Response through NF-κB Signaling.

Soria-Valles C, Gutiérrez-Fernández A, Osorio FG, Carrero D, Ferrando AA, Colado E, Fernández-García MS, Bonzon-Kulichenko E, Vázquez J, Fueyo A, López-Otín C.

J Immunol. 2016 Jul 1;197(1):296-302. doi: 10.4049/jimmunol.1600094. Epub 2016 Jun 3.


Proteostasis alterations in myeloproliferative neoplasms: Oncogenic relevance and therapeutic opportunities.

Santiago-Fernández O, Osorio FG, López-Otín C.

Exp Hematol. 2016 Jul;44(7):574-7. doi: 10.1016/j.exphem.2016.04.004. Epub 2016 Apr 14. Review.


The novel tumor suppressor AIRAPL regulates IGF1R proteostasis.

Osorio FG, Freije JM, López-Otín C.

Cell Cycle. 2016;15(7):873-4. doi: 10.1080/15384101.2016.1151725. Epub 2016 Feb 22. No abstract available.


Loss of the proteostasis factor AIRAPL causes myeloid transformation by deregulating IGF-1 signaling.

Osorio FG, Soria-Valles C, Santiago-Fernández O, Bernal T, Mittelbrunn M, Colado E, Rodríguez F, Bonzon-Kulichenko E, Vázquez J, Porta-de-la-Riva M, Cerón J, Fueyo A, Li J, Green AR, Freije JM, López-Otín C.

Nat Med. 2016 Jan;22(1):91-6. doi: 10.1038/nm.4013. Epub 2015 Dec 21.


Reprogramming aging through DOT1L inhibition.

Soria-Valles C, Osorio FG, López-Otín C.

Cell Cycle. 2015;14(21):3345-6. doi: 10.1080/15384101.2015.1093443. No abstract available.


NF-κB activation impairs somatic cell reprogramming in ageing.

Soria-Valles C, Osorio FG, Gutiérrez-Fernández A, De Los Angeles A, Bueno C, Menéndez P, Martín-Subero JI, Daley GQ, Freije JM, López-Otín C.

Nat Cell Biol. 2015 Aug;17(8):1004-13. doi: 10.1038/ncb3207. Epub 2015 Jul 27. Retraction in: Nat Cell Biol. 2019 Mar;21(3):410.


Loss of MT1-MMP causes cell senescence and nuclear defects which can be reversed by retinoic acid.

Gutiérrez-Fernández A, Soria-Valles C, Osorio FG, Gutiérrez-Abril J, Garabaya C, Aguirre A, Fueyo A, Fernández-García MS, Puente XS, López-Otín C.

EMBO J. 2015 Jul 14;34(14):1875-88. doi: 10.15252/embj.201490594. Epub 2015 May 19.


Loss of GLUT4 induces metabolic reprogramming and impairs viability of breast cancer cells.

Garrido P, Osorio FG, Morán J, Cabello E, Alonso A, Freije JM, González C.

J Cell Physiol. 2015 Jan;230(1):191-8. doi: 10.1002/jcp.24698.


Nuclear envelope lamin-A couples actin dynamics with immunological synapse architecture and T cell activation.

González-Granado JM, Silvestre-Roig C, Rocha-Perugini V, Trigueros-Motos L, Cibrián D, Morlino G, Blanco-Berrocal M, Osorio FG, Freije JMP, López-Otín C, Sánchez-Madrid F, Andrés V.

Sci Signal. 2014 Apr 22;7(322):ra37. doi: 10.1126/scisignal.2004872.


Identification of mitochondrial dysfunction in Hutchinson-Gilford progeria syndrome through use of stable isotope labeling with amino acids in cell culture.

Rivera-Torres J, Acín-Perez R, Cabezas-Sánchez P, Osorio FG, Gonzalez-Gómez C, Megias D, Cámara C, López-Otín C, Enríquez JA, Luque-García JL, Andrés V.

J Proteomics. 2013 Oct 8;91:466-77. doi: 10.1016/j.jprot.2013.08.008. Epub 2013 Aug 20.


Prelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion.

de la Rosa J, Freije JM, Cabanillas R, Osorio FG, Fraga MF, Fernández-García MS, Rad R, Fanjul V, Ugalde AP, Liang Q, Prosser HM, Bradley A, Cadiñanos J, López-Otín C.

Nat Commun. 2013;4:2268. doi: 10.1038/ncomms3268.


Defective extracellular pyrophosphate metabolism promotes vascular calcification in a mouse model of Hutchinson-Gilford progeria syndrome that is ameliorated on pyrophosphate treatment.

Villa-Bellosta R, Rivera-Torres J, Osorio FG, Acín-Pérez R, Enriquez JA, López-Otín C, Andrés V.

Circulation. 2013 Jun 18;127(24):2442-51. doi: 10.1161/CIRCULATIONAHA.112.000571. Epub 2013 May 20.


Luminescence-based in vivo monitoring of NF-κB activity through a gene delivery approach.

Osorio FG, de la Rosa J, Freije JM.

Cell Commun Signal. 2013 Mar 21;11(1):19. doi: 10.1186/1478-811X-11-19.


Detection of nuclear envelope alterations in senescence.

Bárcena C, Osorio FG, Freije JM.

Methods Mol Biol. 2013;965:243-51. doi: 10.1007/978-1-62703-239-1_16.


NF-kB in premature aging.

Osorio FG, López-Otín C, Freije JM.

Aging (Albany NY). 2012 Nov;4(11):726-7. Review. No abstract available.


Nuclear lamina defects cause ATM-dependent NF-κB activation and link accelerated aging to a systemic inflammatory response.

Osorio FG, Bárcena C, Soria-Valles C, Ramsay AJ, de Carlos F, Cobo J, Fueyo A, Freije JM, López-Otín C.

Genes Dev. 2012 Oct 15;26(20):2311-24. doi: 10.1101/gad.197954.112. Epub 2012 Sep 26.


Cell autonomous and systemic factors in progeria development.

Osorio FG, Ugalde AP, Mariño G, Puente XS, Freije JM, López-Otín C.

Biochem Soc Trans. 2011 Dec;39(6):1710-4. doi: 10.1042/BST20110677. Review.


Splicing-directed therapy in a new mouse model of human accelerated aging.

Osorio FG, Navarro CL, Cadiñanos J, López-Mejía IC, Quirós PM, Bartoli C, Rivera J, Tazi J, Guzmán G, Varela I, Depetris D, de Carlos F, Cobo J, Andrés V, De Sandre-Giovannoli A, Freije JM, Lévy N, López-Otín C.

Sci Transl Med. 2011 Oct 26;3(106):106ra107. doi: 10.1126/scitranslmed.3002847.


A conserved splicing mechanism of the LMNA gene controls premature aging.

Lopez-Mejia IC, Vautrot V, De Toledo M, Behm-Ansmant I, Bourgeois CF, Navarro CL, Osorio FG, Freije JM, Stévenin J, De Sandre-Giovannoli A, Lopez-Otin C, Lévy N, Branlant C, Tazi J.

Hum Mol Genet. 2011 Dec 1;20(23):4540-55. doi: 10.1093/hmg/ddr385. Epub 2011 Aug 29.


Germ-line mutations in epidermal growth factor receptor (EGFR) are rare but may contribute to oncogenesis: a novel germ-line mutation in EGFR detected in a patient with lung adenocarcinoma.

Centeno I, Blay P, Santamaría I, Astudillo A, Pitiot AS, Osorio FG, González-Arriaga P, Iglesias F, Menéndez P, Tardón A, Freije JM, Balbín M.

BMC Cancer. 2011 May 16;11:172. doi: 10.1186/1471-2407-11-172.


Exome sequencing and functional analysis identifies BANF1 mutation as the cause of a hereditary progeroid syndrome.

Puente XS, Quesada V, Osorio FG, Cabanillas R, Cadiñanos J, Fraile JM, Ordóñez GR, Puente DA, Gutiérrez-Fernández A, Fanjul-Fernández M, Lévy N, Freije JM, López-Otín C.

Am J Hum Genet. 2011 May 13;88(5):650-6. doi: 10.1016/j.ajhg.2011.04.010. Epub 2011 May 5.


Nuclear envelope alterations generate an aging-like epigenetic pattern in mice deficient in Zmpste24 metalloprotease.

Osorio FG, Varela I, Lara E, Puente XS, Espada J, Santoro R, Freije JM, Fraga MF, López-Otín C.

Aging Cell. 2010 Dec;9(6):947-57. doi: 10.1111/j.1474-9726.2010.00621.x. Epub 2010 Oct 21.


Insulin-like growth factor 1 treatment extends longevity in a mouse model of human premature aging by restoring somatotroph axis function.

Mariño G, Ugalde AP, Fernández AF, Osorio FG, Fueyo A, Freije JM, López-Otín C.

Proc Natl Acad Sci U S A. 2010 Sep 14;107(37):16268-73. doi: 10.1073/pnas.1002696107. Epub 2010 Aug 30.


Accelerated ageing: from mechanism to therapy through animal models.

Osorio FG, Obaya AJ, López-Otín C, Freije JM.

Transgenic Res. 2009 Feb;18(1):7-15. doi: 10.1007/s11248-008-9226-z. Epub 2008 Nov 18. Review.


Combined treatment with statins and aminobisphosphonates extends longevity in a mouse model of human premature aging.

Varela I, Pereira S, Ugalde AP, Navarro CL, Suárez MF, Cau P, Cadiñanos J, Osorio FG, Foray N, Cobo J, de Carlos F, Lévy N, Freije JM, López-Otín C.

Nat Med. 2008 Jul;14(7):767-72. doi: 10.1038/nm1786. Epub 2008 Jun 29.


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