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J Clin Invest. 2017 Feb 1;127(2):405-414. doi: 10.1172/JCI87440. Epub 2017 Feb 1.

Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic age-related lung disease with high mortality that is characterized by abnormal scarring of the lung parenchyma. There has been a recent attempt to define the age-associated changes predisposing individuals to develop IPF. Age-related perturbations that are increasingly found in epithelial cells and fibroblasts from IPF lungs compared with age-matched cells from normal lungs include defective autophagy, telomere attrition, altered proteostasis, and cell senescence. These divergent processes seem to converge in mitochondrial dysfunction and metabolic distress, which potentiate maladaptation to stress and susceptibility to age-related diseases such as IPF. Therapeutic approaches that target aging processes may be beneficial for halting the progression of disease and improving quality of life in IPF patients.

PMID:
28145905
PMCID:
PMC5272191
DOI:
10.1172/JCI87440
[Indexed for MEDLINE]
Free PMC Article

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