Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/10312
Título
Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction.
Autor(es)
Fecha de publicación
2015-07-14
Cita
Cell Rep. 2015;12(2):286-99.
Idioma
Inglés
Tipo de documento
journal article
Resumen
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.
MESH
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
Versión en línea
DOI
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