Idiopathic pulmonary fibrosis (IPF) is a significant health concern, especially among the elderly, given its chronic and progressive nature, unknown origins, and limited life expectancy post-diagnosis. Current understanding of IPF suggests that the disease originates from recurrent injury to the alveolar epithelial cells, resulting in excessive and aberrant extracellular matrix (ECM) deposition in the alveolar interstitium by fibroblasts. This excessive ECM destroys alveoli, impairing lung function and ultimately leading to fatality.
Recent evidence suggests a surge in IPF incidence, particularly post-pandemic, and the limited effectiveness of available antifibrotic drugs underscores the critical need for a deeper understanding of the disease biology to design more effective therapeutic strategies. Animal and ex vivo models have significantly contributed to comprehending complex cellular and molecular pathways involved in fibrosis progression. Therefore, this Methods Collection aims to provide invaluable resources for the research community by offering standardized protocols for preclinical models developed by experts to advance our understanding of fibrosis pathogenesis.
The collection serves as a comprehensive guide for generating animal models, utilizing ex vivo models, and conducting qualitative and quantitative assays. It places special emphasis on analyzing disease severity through collagen content quantification and in vivo image-based analysis. By compiling these methodologies, the collection facilitates rigorous and reproducible research, ultimately driving progress toward more effective therapies for IPF.
0 Views
•
Cited by 2
•
2025
•
1Department of Medicine, Division of Pulmonary & Critical Care, David Geffen School of Medicine, University of California, Los Angeles, 2Department of Medicine, Pulmonary & Critical Care Section, VA Greater Los Angeles Healthcare System, 3Department of Microbiology, Immunology and Molecular Genetics, University of California, Los Angeles