Journal
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY
Volume 324, Issue 6, Pages L737-L746Publisher
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/ajplung.00229.2022
Keywords
fi brosis; IPF; lineage tracing; lung; lung fi brosis; mouse models; myo fi broblasts
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Idiopathic pulmonary fibrosis (IPF) is a rare interstitial lung disease with a poor prognosis. The origin of fibrosis-associated myofibroblasts in pulmonary fibrosis is not fully understood to date.
Idiopathic pulmonary fibrosis (IPF) is a rare interstitial lung disease with a poor prognosis. Chronic microinjuries, mainly caused by environmental factors to an aging alveolar epithelium, would lead to the aberrant differentiation and accumulation of aberrant mesenchymal cells with a contractile phenotype, known as fibrosis-associated myofibroblasts, which trigger abnormal extracellu-lar matrix accumulation and fibrosis. The origin of those pathological myofibroblasts in pulmonary fibrosis is not fully understood to date. Lineage tracing methods using mouse models have opened new avenues for studying cell fate in a pathological con-text. This review aims to present a nonexhaustive list of different potential sources of those harmful myofibroblasts during lung fi- brosis, based on these in vivo approaches, and considering the normal and fibrotic lung cellular atlas recently established by single-cell RNA sequencing.
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