The contribution of animal models to understanding the role of the immune system in human idiopathic pulmonary fibrosis

Clinical & Translational Immunology

Mark W Fear¹  Gerard F Hoyne²  Cecilia M Prêle³  Tylah Miles⁴  Steven E Mutsaers⁴  Darryl A Knight⁵ 

[1] Burn Injury Research Unit School of Biomedical Sciences The University of Western Australia Crawley WA Australia;Centre for Cell Therapy and Regenerative Medicine School of Biomedical Sciences University of Western Australia Nedlands WA Australia;Centre for Respiratory Health School of Biomedical Sciences University of Western Australia Nedlands WA Australia;Institute for Respiratory Health Nedlands WA Australia;Providence Health Care Research Institute Vancouver BC Canada;

关键词: animal models;    bleomycin;    fibrogenesis;    inflammation;    innate and adaptive immune system;   

DOI  :  10.1002/cti2.1153

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown