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oai:repisalud.isciii.es:20.500.12105/181812024-11-29T21:05:19Zcom_20.500.12105_2173com_20.500.12105_2051col_20.500.12105_19597

00925njm 22002777a 4500 dc Piñeiro-Hermida, Sergio author Autilio, Chiara author Martínez, Paula author Bosch, Fátima author Pérez-Gil, Jesús author Blasco, MA author MARTINEZ author 2020-10-05 Short/dysfunctional telomeres are at the origin of idiopathic pulmonary fibrosis (IPF) in patients mutant for telomere maintenance genes. However, it remains unknown whether physiological aging leads to short telomeres in the lung, thus leading to IPF with aging. Here, we find that physiological aging in wild-type mice leads to telomere shortening and a reduced proliferative potential of alveolar type II cells and club cells, increased cellular senescence and DNA damage, increased fibroblast activation and collagen deposits, and impaired lung biophysics, suggestive of a fibrosis-like pathology. Treatment of both wild-type and telomerase-deficient mice with telomerase gene therapy prevented the onset of lung profibrotic pathologies. These findings suggest that short telomeres associated with physiological aging are at the origin of IPF and that a potential treatment for IPF based on telomerase activation would be of interest not only for patients with telomerase mutations but also for sporadic cases of IPF associated with physiological aging. J Cell Biol . 2020 ;219(10):e202002120. 10.1083/jcb.202002120 1540-8140 The Journal of cell biology 32777016 http://hdl.handle.net/20.500.12105/18181 Telomerase treatment prevents lung profibrotic pathologies associated with physiological aging.