Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.

Idiopathic 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 protectio...

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Detalles Bibliográficos
Autores: Flores, Juana M, Blasco, MA, Mulero, Francisca, Martinez Rodriguez, Paula
Tipo de recurso: artículo
Fecha de publicación:2015
País:España
Institución:Instituto de Salud Carlos III (ISCIII)
Repositorio:Repisalud
Idioma:inglés
OAI Identifier:oai:repisalud.isciii.es:20.500.12105/10312
Acceso en línea:http://hdl.handle.net/20.500.12105/10312
Access Level:acceso abierto
Palabra clave:Animals
Cyclin-Dependent Kinase Inhibitor p21
DNA Damage
DNA Repair
Disease Models, Animal
Female
Idiopathic Pulmonary Fibrosis
Lung
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Radiography
Tamoxifen
Telomerase
Telomere
Telomere Shortening
Telomeric Repeat Binding Protein 1
Failure
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oai_identifier_str oai:repisalud.isciii.es:20.500.12105/10312
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repository_id_str
spelling Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.Flores, Juana MBlasco, MAMulero, FranciscaMartinez Rodriguez, PaulaAnimalsCyclin-Dependent Kinase Inhibitor p21DNA DamageDNA RepairDisease Models, AnimalFemaleIdiopathic Pulmonary FibrosisLungMaleMiceMice, Inbred C57BLMice, KnockoutRadiographyTamoxifenTelomeraseTelomereTelomere ShorteningTelomeric Repeat Binding Protein 1FailureIdiopathic 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.Cell PressMinisterio de Economía y Competitividad (España)Unión EuropeaUnión Europea. Comisión Europea. European Research Council (ERC)Korber FoundationFundación AXABotín FoundationFundación Lilly20202020-06-0920152015-07-1420152015-07-14journal articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfapplication/pdfapplication/pdfhttp://hdl.handle.net/20.500.12105/10312reponame:Repisaludinstname:Instituto de Salud Carlos III (ISCIII)Inglésengopen accesshttp://purl.org/coar/access_right/c_abf2Atribución-NoComercial-CompartirIgual 4.0 Internacionalhttp://creativecommons.org/licenses/by-nc-sa/4.0/info:eu-repo/semantics/openAccessoai:repisalud.isciii.es:20.500.12105/103122026-06-12T12:43:37Z
dc.title.none.fl_str_mv Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
title Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
spellingShingle Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
Flores, Juana M
Animals
Cyclin-Dependent Kinase Inhibitor p21
DNA Damage
DNA Repair
Disease Models, Animal
Female
Idiopathic Pulmonary Fibrosis
Lung
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Radiography
Tamoxifen
Telomerase
Telomere
Telomere Shortening
Telomeric Repeat Binding Protein 1
Failure
title_short Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
title_full Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
title_fullStr Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
title_full_unstemmed Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
title_sort Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
dc.creator.none.fl_str_mv Flores, Juana M
Blasco, MA
Mulero, Francisca
Martinez Rodriguez, Paula
author Flores, Juana M
author_facet Flores, Juana M
Blasco, MA
Mulero, Francisca
Martinez Rodriguez, Paula
author_role author
author2 Blasco, MA
Mulero, Francisca
Martinez Rodriguez, Paula
author2_role author
author
author
dc.contributor.none.fl_str_mv Ministerio de Economía y Competitividad (España)
Unión Europea
Unión Europea. Comisión Europea. European Research Council (ERC)
Korber Foundation
Fundación AXA
Botín Foundation
Fundación Lilly
dc.subject.none.fl_str_mv Animals
Cyclin-Dependent Kinase Inhibitor p21
DNA Damage
DNA Repair
Disease Models, Animal
Female
Idiopathic Pulmonary Fibrosis
Lung
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Radiography
Tamoxifen
Telomerase
Telomere
Telomere Shortening
Telomeric Repeat Binding Protein 1
Failure
topic Animals
Cyclin-Dependent Kinase Inhibitor p21
DNA Damage
DNA Repair
Disease Models, Animal
Female
Idiopathic Pulmonary Fibrosis
Lung
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Radiography
Tamoxifen
Telomerase
Telomere
Telomere Shortening
Telomeric Repeat Binding Protein 1
Failure
description Idiopathic 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.
publishDate 2015
dc.date.none.fl_str_mv 2015
2015-07-14
2015
2015-07-14
2020
2020-06-09
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
VoR
http://purl.org/coar/version/c_970fb48d4fbd8a85
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv http://hdl.handle.net/20.500.12105/10312
url http://hdl.handle.net/20.500.12105/10312
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
Atribución-NoComercial-CompartirIgual 4.0 Internacional
http://creativecommons.org/licenses/by-nc-sa/4.0/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
Atribución-NoComercial-CompartirIgual 4.0 Internacional
http://creativecommons.org/licenses/by-nc-sa/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
application/pdf
application/pdf
dc.publisher.none.fl_str_mv Cell Press
publisher.none.fl_str_mv Cell Press
dc.source.none.fl_str_mv reponame:Repisalud
instname:Instituto de Salud Carlos III (ISCIII)
instname_str Instituto de Salud Carlos III (ISCIII)
reponame_str Repisalud
collection Repisalud
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869408691782942721
score 15,812429

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