Antonella Marrocco will present on the paper: "Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction"
EOH Journal Club Seminar - Spring 2016 Date: Thursday January 28, 2016 Time: 11am - 12pm Presenter: Antonella Marrocco Paper: Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction Authors: Povedano JM, Martinez P, Flores JM, Mulero F, Blasco MA Abstract: 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.