Journal article

Two-Way Conversion between Lipogenic and Myogenic Fibroblastic Phenotypes Marks the Progression and Resolution of Lung Fibrosis


Authors listEl Agha, Elie; Moiseenko, Alena; Kheirollahi, Vahid; De langhe, Stijn; Crnkovic, Slaven; Kwapiszewska, Grazyna; Kosanovic, Djuro; Schwind, Felix; Schermuly, Ralph T.; Henneke, Ingrid; MacKenzie, BreAnne; Quantius, Jennifer; Herold, Susanne; Ntokou, Aglaia; Ahlbrecht, Katrin; Morty, Rory E.; Guenther, Andreas; Seeger, Werner; Bellusci, Saverio

Publication year2017

Pages261-26+

JournalCell Stem Cell

Volume number20

Issue number2

ISSN1934-5909

eISSN1875-9777

Open access statusGreen

DOI Linkhttps://doi.org/10.1016/j.stem.2016.10.004

PublisherElsevier


Abstract
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.



Citation Styles

Harvard Citation styleEl Agha, E., Moiseenko, A., Kheirollahi, V., De langhe, S., Crnkovic, S., Kwapiszewska, G., et al. (2017) Two-Way Conversion between Lipogenic and Myogenic Fibroblastic Phenotypes Marks the Progression and Resolution of Lung Fibrosis, Cell Stem Cell, 20(2), pp. 261-26+. https://doi.org/10.1016/j.stem.2016.10.004

APA Citation styleEl Agha, E., Moiseenko, A., Kheirollahi, V., De langhe, S., Crnkovic, S., Kwapiszewska, G., Kosanovic, D., Schwind, F., Schermuly, R., Henneke, I., MacKenzie, B., Quantius, J., Herold, S., Ntokou, A., Ahlbrecht, K., Morty, R., Guenther, A., Seeger, W., & Bellusci, S. (2017). Two-Way Conversion between Lipogenic and Myogenic Fibroblastic Phenotypes Marks the Progression and Resolution of Lung Fibrosis. Cell Stem Cell. 20(2), 261-26+. https://doi.org/10.1016/j.stem.2016.10.004



Keywords


FIBROBLAST-GROWTH-FACTOR-10FIBROCYTESLIPOFIBROBLASTMYOFIBROBLASTPULMONARY-FIBROSISSMOOTH-MUSCLE-CELLS

Last updated on 2025-10-06 at 10:43