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- Title
The contribution of animal models to understanding the role of the immune system in human idiopathic pulmonary fibrosis.
- Authors
Miles, Tylah; Hoyne, Gerard F; Knight, Darryl A; Fear, Mark W; Mutsaers, Steven E; Prêle, Cecilia M
- Abstract
Pulmonary fibrosis occurs in a heterogeneous group of lung disorders and is characterised by an excessive deposition of extracellular matrix proteins within the pulmonary interstitium, leading to impaired gas transfer and a loss of lung function. In the past 10 years, there has been a dramatic increase in our understanding of the immune system and how it contributes to fibrogenic processes within the lung. This review will compare some of the models used to investigate the pathogenesis and treatment of pulmonary fibrosis, in particular those used to study immune cell pathogenicity in idiopathic pulmonary fibrosis, highlighting their advantages and disadvantages in dissecting human disease.
- Subjects
IDIOPATHIC pulmonary fibrosis; FIBROSIS; IMMUNE system; EXTRACELLULAR matrix proteins; PULMONARY fibrosis; ANIMAL models in research
- Publication
Clinical & Translational Immunology, 2020, Vol 9, Issue 7, p1
- ISSN
2050-0068
- Publication type
Article
- DOI
10.1002/cti2.1153