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Lung organoids and other preclinical models of pulmonary fibrosis
- 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.
- Subjects :
- Disease
Extracellular matrix
03 medical and health sciences
Idiopathic pulmonary fibrosis
0302 clinical medicine
Pulmonary fibrosis
Animals
Humans
Medicine
Induced pluripotent stem cell
Lung
030304 developmental biology
0303 health sciences
business.industry
General Medicine
Fibroblasts
respiratory system
medicine.disease
Idiopathic Pulmonary Fibrosis
Europe
Organoids
medicine.anatomical_structure
030228 respiratory system
Drug development
Quality of Life
Cancer research
business
Ex vivo
Subjects
Details
- ISSN :
- 14602393 and 14602725
- Database :
- OpenAIRE
- Journal :
- QJM: An International Journal of Medicine
- Accession number :
- edsair.doi.dedup.....30368705205314389bd73ba95ea17854