The DOI matches its Crossref record; 157 of the 157 references it cites were found in a registry or an index.
Contributions of alveolar epithelial cell quality control to pulmonary fibrosis
Open accessAbstract
Epithelial cell dysfunction has emerged as a central component of the pathophysiology of diffuse parenchymal diseases including idiopathic pulmonary fibrosis (IPF). Alveolar type 2 (AT2) cells represent a metabolically active lung cell population important for surfactant biosynthesis and alveolar homeostasis. AT2 cells and other distal lung epithelia, like all eukaryotic cells, contain an elegant quality control network to respond to intrinsic metabolic and biosynthetic challenges imparted by mutant protein conformers, dysfunctional subcellular organelles, and dysregulated telomeres. Failed AT2 quality control components (the ubiquitin-proteasome system, unfolded protein response, macroautophagy, mitophagy, and telomere maintenance) result in diverse cellular endophenotypes and molecular signatures including ER stress, defective autophagy, mitochondrial dysfunction, apoptosis, inflammatory cell recruitment, profibrotic signaling, and altered progenitor function that ultimately converge to drive downstream fibrotic remodeling in the IPF lung. As this complex network becomes increasingly better understood, opportunities will emerge to identify targets and therapeutic strategies for IPF.
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1 retracted
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BackgroundEfficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
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Mitochondrial Fission, Fusion, and Stress
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Cited by
Ruaro, B., Salton, F., Braga, L., Wade, B., Confalonieri, P., Volpe, M. C., Baratella, E., Maiocchi, S., & Confalonieri, M. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22052566 · Cited by 300
Inhibition of ferroptosis and iron accumulation alleviates pulmonary fibrosis in a bleomycin model
Pei, Z., Qin, Y., Fu, X., Yang, F., Huo, F., Liang, X., Wang, S., Cui, H., Lin, P., Zhou, G., et al. (2022) · Redox Biology · doi:10.1016/j.redox.2022.102509 · Cited by 269
The Hedgehog Signaling Pathway in Idiopathic Pulmonary Fibrosis: Resurrection Time
Effendi, W. I., & Nagano, T. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms23010171 · Cited by 59
Metadata
- Authors
- Katzen, J., & Beers, M. F.
- Venue
- Journal of Clinical Investigation
- Published
- 2020-09-21
- Volume
- 130
- Issue
- 10
- Pages
- 5088-5099
- ISSN
- 0021-9738 (ISSN-L)
- 1558-8238
- OpenAlex
- W3081562004
- Cited by
- 287
- References
- 157
- FWCI
- 16.62
- Type
- journal article
- Language
- en
- Open access
- bronze
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
