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Usage Information

Contributions of alveolar epithelial cell quality control to pulmonary fibrosis
Jeremy Katzen, Michael F. Beers
Jeremy Katzen, Michael F. Beers
Published September 1, 2020
Citation Information: J Clin Invest. 2020;130(10):5088-5099. https://doi.org/10.1172/JCI139519.
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Review

Contributions of alveolar epithelial cell quality control to pulmonary fibrosis

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Abstract

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.

Authors

Jeremy Katzen, Michael F. Beers

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Usage data is cumulative from July 2024 through July 2025.

Usage JCI PMC
Text version 3,446 1,061
PDF 239 269
Figure 414 4
Table 73 0
Citation downloads 112 0
Totals 4,284 1,334
Total Views 5,618
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Usage information is collected from two different sources: this site (JCI) and Pubmed Central (PMC). JCI information (compiled daily) shows human readership based on methods we employ to screen out robotic usage. PMC information (aggregated monthly) is also similarly screened of robotic usage.

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