Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia, and it has a worse prognosis than non-small cell lung cancer. The pathomechanism of IPF is not fully understood, but it has been suggested that repeated microinjuries of epithelial cells induce a wound...
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MDPI AG
2022-05-01
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author | I-Chen Chen Yi-Ching Liu Yen-Hsien Wu Shih-Hsing Lo Zen-Kong Dai Jong-Hau Hsu Yu-Hsin Tseng |
author_facet | I-Chen Chen Yi-Ching Liu Yen-Hsien Wu Shih-Hsing Lo Zen-Kong Dai Jong-Hau Hsu Yu-Hsin Tseng |
author_sort | I-Chen Chen |
collection | DOAJ |
description | Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia, and it has a worse prognosis than non-small cell lung cancer. The pathomechanism of IPF is not fully understood, but it has been suggested that repeated microinjuries of epithelial cells induce a wound healing response, during which fibroblasts differentiate into myofibroblasts. These activated myofibroblasts express α smooth muscle actin and release extracellular matrix to promote matrix deposition and tissue remodeling. Under physiological conditions, the remodeling process stops once wound healing is complete. However, in the lungs of IPF patients, myofibroblasts re-main active and deposit excess extracellular matrix. This leads to the destruction of alveolar tissue, the loss of lung elastic recoil, and a rapid decrease in lung function. Some evidence has indicated that proteasomal inhibition combats fibrosis by inhibiting the expressions of extracellular matrix proteins and metalloproteinases. However, the mechanisms by which proteasome inhibitors may protect against fibrosis are not known. This review summarizes the current research on proteasome inhibitors for pulmonary fibrosis, and provides a reference for whether proteasome inhibitors have the potential to become new drugs for the treatment of pulmonary fibrosis. |
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issn | 2073-4409 |
language | English |
last_indexed | 2024-03-10T04:15:47Z |
publishDate | 2022-05-01 |
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spelling | doaj.art-383b0cc1ed8c4950a0b6b3d7c99c61d92023-11-23T08:00:46ZengMDPI AGCells2073-44092022-05-01119154310.3390/cells11091543Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary FibrosisI-Chen Chen0Yi-Ching Liu1Yen-Hsien Wu2Shih-Hsing Lo3Zen-Kong Dai4Jong-Hau Hsu5Yu-Hsin Tseng6Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanDepartment of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, TaiwanIdiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia, and it has a worse prognosis than non-small cell lung cancer. The pathomechanism of IPF is not fully understood, but it has been suggested that repeated microinjuries of epithelial cells induce a wound healing response, during which fibroblasts differentiate into myofibroblasts. These activated myofibroblasts express α smooth muscle actin and release extracellular matrix to promote matrix deposition and tissue remodeling. Under physiological conditions, the remodeling process stops once wound healing is complete. However, in the lungs of IPF patients, myofibroblasts re-main active and deposit excess extracellular matrix. This leads to the destruction of alveolar tissue, the loss of lung elastic recoil, and a rapid decrease in lung function. Some evidence has indicated that proteasomal inhibition combats fibrosis by inhibiting the expressions of extracellular matrix proteins and metalloproteinases. However, the mechanisms by which proteasome inhibitors may protect against fibrosis are not known. This review summarizes the current research on proteasome inhibitors for pulmonary fibrosis, and provides a reference for whether proteasome inhibitors have the potential to become new drugs for the treatment of pulmonary fibrosis.https://www.mdpi.com/2073-4409/11/9/1543idiopathic pulmonary fibrosisproteasome inhibitortransforming growth factor-beta |
spellingShingle | I-Chen Chen Yi-Ching Liu Yen-Hsien Wu Shih-Hsing Lo Zen-Kong Dai Jong-Hau Hsu Yu-Hsin Tseng Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis Cells idiopathic pulmonary fibrosis proteasome inhibitor transforming growth factor-beta |
title | Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis |
title_full | Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis |
title_fullStr | Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis |
title_full_unstemmed | Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis |
title_short | Evaluation of Proteasome Inhibitors in the Treatment of Idiopathic Pulmonary Fibrosis |
title_sort | evaluation of proteasome inhibitors in the treatment of idiopathic pulmonary fibrosis |
topic | idiopathic pulmonary fibrosis proteasome inhibitor transforming growth factor-beta |
url | https://www.mdpi.com/2073-4409/11/9/1543 |
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