期刊
CELL STEM CELL
卷 20, 期 2, 页码 261-+出版社
CELL PRESS
DOI: 10.1016/j.stem.2016.10.004
关键词
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资金
- Excellence Cluster CardioPulmonary System (ECCPS)
- Universitatsklinikum Giessen und Marburg (UKGM)
- Deutsche Forschungsgemeinschaft (DFG) [BE4443/4-1, BE4443/6-1, CRC1213]
- Landes-Offensive zur Entwicklung Wissenschaftlich-okonomischer Exzellenz (LOEWE)
- UKGM
- Universities of Giessen and Marburg Lung Center (UGMLC)
- German Center for Lung Research (DZL)
- COST [BM1201]
- NIH/NHLBI [1R01HL086322-01A2, HL107307, R01HL126732, R01HL132156]
- DZL
- ECCPS
- UGMLC
- DFG [SFB1021, SFB TR84]
- UKGM (FOKOOPV)
- March of Dimes [1-FY15-352]
- pulmonary fibrosis foundation (The Albert Rose established investigator award)
Idiopathic pulmonary fibrosis (IPF) is a form of progressive interstitial lung disease with unknown etiology. Due to a lack of effective treatment, IPF is associated with a high mortality rate. The hallmark feature of this disease is the accumulation of activated myofibroblasts that excessively deposit extracellular matrix proteins, thus compromising lung architecture and function and hindering gas exchange. Here we investigated the origin of activated myofibroblasts and the molecular mechanisms governing fibrosis formation and resolution. Genetic engineering in mice enables the time-controlled labeling and monitoring of lipogenic or myogenic populations of lung fibroblasts during fibrosis formation and resolution. Our data demonstrate a lipogenic-to-myogenic switch in fibroblastic phenotype during fibrosis formation. Conversely, we observed a myogenic-to-lipogenic switch during fibrosis resolution. Analysis of human lung tissues and primary human lung fibroblasts indicates that this fate switching is involved in IPF pathogenesis, opening potential therapeutic avenues to treat patients.
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