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Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis
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Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis
Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis
Journal Article

Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis

2016
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Overview
Idiopathic pulmonary fibrosis (IPF) is an aging-associated, progressive, and irreversible lung disease of unknown etiology, elusive pathogenesis, and very limited therapeutic options. The hallmarks of IPF are aberrant activation of alveolar epithelial cells and accumulation of fibroblasts and myofibroblasts along with excessive production of extracellular matrix. The linkage of aging with this disorder is uncertain, but a number of changes associated with aging, including telomere attrition, cell senescence, and mitochondrial dysfunction, have been revealed in IPF lungs. Also, aging seems to confer a profibrotic phenotype upon fibroblasts and to increase the severity of the fibrogenic response in non-IPF fibrotic lung disorders. Better knowledge of the pathophysiological mechanisms linking aging to IPF will advance understanding of its pathogenesis and may provide new therapeutic windows to treatment of this devastating disease.