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Lung organoids and other preclinical models of pulmonary fibrosis

Authors :
Irene Oglesby
Anja Schweikert
Seamas C. Donnelly
Killian Hurley
Brian Fox
Cliodhna Redmond
Source :
QJM: An International Journal of Medicine.
Publication Year :
2021
Publisher :
Oxford University Press (OUP), 2021.

Abstract

Summary Idiopathic pulmonary fibrosis (IPF) is a progressive fatal disease affecting over 100 000 people in Europe with an increasing incidence. Available treatments offer only slowing of disease progression and are poorly tolerated by patients leading to cessation of therapy. Lung transplant remains the only cure. Therefore, alternative treatments are urgently required. The pathology of IPF is complex and poorly understood and thus creates a major obstacle to the discovery of novel treatments. Additionally, preclinical assessment of new treatments currently relies upon animal models where disparities with human lung biology often hamper drug development. At a cellular level, IPF is characterized by persistent and abnormal deposition of extracellular matrix by fibroblasts and alveolar epithelial cell injury which is seen as a key event in initiation of disease progression. In-depth investigation of the role of alveolar epithelial cells in health and disease has been impeded due to difficulties in primary cell isolation and culture ex vivo. Novel strategies employing patient-derived induced pluripotent stem cells engineered to produce type 2 alveolar epithelial cells (iAEC2) cultured as three-dimensional organoids have the potential to overcome these hurdles and inform new effective precision treatments for IPF leading to improved survival and quality of life for patients worldwide.

Details

ISSN :
14602393 and 14602725
Database :
OpenAIRE
Journal :
QJM: An International Journal of Medicine
Accession number :
edsair.doi.dedup.....30368705205314389bd73ba95ea17854