کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2039377 1073052 2015 14 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Mice with Pulmonary Fibrosis Driven by Telomere Dysfunction
ترجمه فارسی عنوان
موش های فیبروز ریه تحت درمان با اختلال تلومر
موضوعات مرتبط
علوم زیستی و بیوفناوری علوم کشاورزی و بیولوژیک علوم کشاورزی و بیولوژیک (عمومی)
چکیده انگلیسی


• Trf1 deletion alone in alveolar type II cells induces pulmonary fibrosis in mice
• Short telomeres and low-dose bleomycin induce pulmonary fibrosis in mice
• These mouse models are instrumental for the development of new therapeutic strategies

SummaryIdiopathic 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.

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ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: - Volume 12, Issue 2, 14 July 2015, Pages 286–299
نویسندگان
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