Epithelial Injury and Dysfunction in the Pathogenesis of Idiopathic PulmonaryFibrosis.
Epithelial Injury and Dysfunction in the Pathogenesis of Idiopathic PulmonaryFibrosis.
Am J Med Sci. 2019 May;357(5):374-378
Authors: Winters NI, Burman A, Kropski JA, Blackwell TS
Abstract
Idiopathic pulmonary fibrosis is a disease of older adults leading to progressive dyspnea and reduced exercise capacity, typically resulting in death within 3-5years of diagnosis. Underlying genetic susceptibility combined with environmental insults is proposed to trigger a chronic wound repair response, leading to activation of the fibrotic cascade. Perturbations in several molecular pathways mediate vulnerability of the alveolar epithelium to injurious agents, including the unfolded protein response, autophagy, mitophagy, and cellular senescence. These cellular responses are intricately intertwined and link genetic susceptibility to the progressive fibrotic phenotype. Ongoing studies investigating these pathways in type II alveolar epithelial cells show promise for identifying new targeted interventions that could prevent or halt the progression of IPF.
PMID: 31010463 [PubMed - in process]
Source: The American Journal of the Medical Sciences - Category: General Medicine Authors: Winters NI, Burman A, Kropski JA, Blackwell TS Tags: Am J Med Sci Source Type: research
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