Regeneration or Repair? The Role of Alveolar Epithelial Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis (IPF)
Abstract
:1. Idiopathic Pulmonary Fibrosis
1.1. Definition and Epidemiology
1.2. Clinical Manifestation and Diagnosis
2. Current Knowledge of Cellular and Molecular Mechanisms in IPF Onset
2.1. Epithelial-to-Mesenchymal Transition as a Nonmesenchymal Source of Fibrosis
2.2. The Role of Bidirectional Epithelial–Mesenchymal Crosstalk in Fibroblast-to-Myofibroblast Activation
2.3. Cellular Senescence and IPF: A Causative Link
2.4. The Role of ECM in IPF Onset and Progression
3. Current Knowledge of Cellular and Molecular Mechanisms in Lung Regeneration
3.1. Lung Embryogenesis and Development
3.2. Alveolar Type II Pneumocytes and Their Response to Lung Injury
4. Current Therapies for IPF
- (1)
- Obtain better mechanistic insights into the interplay between ATII cells and fibroblasts during fibrosis progression, with the aim of identifying novel ATII-related druggable targets for the development of the most effective antifibrotic therapies;
- (2)
- Systematically dissect the molecular mechanisms of ATII-driven physiological lung regeneration in order to find new druggable targets for the development of effective regenerative therapies;
- (3)
- Tackle this disease by simultaneously blocking fibrosis progression and promoting alveolar repair and organ regeneration.
Author Contributions
Funding
Conflicts of Interest
References
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Treatment | Estimated Enrolled Patients | Type | Clinical Trial ID |
---|---|---|---|
Pamrevlumab (FG-3019) | 340 | Phase 3 | NCT03955146 |
PRM-151 | 700 | Phase 3 | NCT04594707–NCT04552899 |
Treprostinil | 396 | Phase 3 | NCT04708782 |
ENV-101 | 60 | Phase 2 | NCT04968574 |
PLN_74809 | 84 | Phase 2 | NCT04396756 |
GKT137831 | 60 | Phase 2 | NCT03865927 |
GB0139 | 500 | Phase 2 | NCT03832946 |
C21 | 60 | Phase 2 | NCT04533022 |
Jaktinib Dihydrochloride Monohydrate | 90 | Phase 2 | NCT04312594 |
Saracatinab | 100 | Phase 1 | NCT04598919 |
ZSP1603 | 36 | Phase 1 | NCT05119972 |
INS018_055 | 80 | Phase 1 | NCT05154240 |
AZD5055 | 104 | Phase 1 | NCT05134727 |
SHR-1906 | 60 | Phase 1 | NCT04986540 |
NIP292 | 72 | Phase 1 | NCT04720443 |
Inhaled Nitric Oxide (iNO) | 40 | Early Phase 1 | NCT05052229 |
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Confalonieri, P.; Volpe, M.C.; Jacob, J.; Maiocchi, S.; Salton, F.; Ruaro, B.; Confalonieri, M.; Braga, L. Regeneration or Repair? The Role of Alveolar Epithelial Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis (IPF). Cells 2022, 11, 2095. https://doi.org/10.3390/cells11132095
Confalonieri P, Volpe MC, Jacob J, Maiocchi S, Salton F, Ruaro B, Confalonieri M, Braga L. Regeneration or Repair? The Role of Alveolar Epithelial Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis (IPF). Cells. 2022; 11(13):2095. https://doi.org/10.3390/cells11132095
Chicago/Turabian StyleConfalonieri, Paola, Maria Concetta Volpe, Justin Jacob, Serena Maiocchi, Francesco Salton, Barbara Ruaro, Marco Confalonieri, and Luca Braga. 2022. "Regeneration or Repair? The Role of Alveolar Epithelial Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis (IPF)" Cells 11, no. 13: 2095. https://doi.org/10.3390/cells11132095
APA StyleConfalonieri, P., Volpe, M. C., Jacob, J., Maiocchi, S., Salton, F., Ruaro, B., Confalonieri, M., & Braga, L. (2022). Regeneration or Repair? The Role of Alveolar Epithelial Cells in the Pathogenesis of Idiopathic Pulmonary Fibrosis (IPF). Cells, 11(13), 2095. https://doi.org/10.3390/cells11132095