Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/16860
Título
Modeling of Respiratory Diseases Evolving with Fibrosis from Organoids Derived from Human Pluripotent Stem Cells
Autor(es)
Chamorro-Herrero, Irene ISCIII | Zambrano, Alberto ISCIII
Fecha de publicación
2023-02-23
Cita
Int J Mol Sci. 2023 Feb 23;24(5):4413.
Idioma
Inglés
Tipo de documento
review article
Resumen
Respiratory disease is one of the leading causes of morbidity and mortality worldwide. There is no cure for most diseases, which are treated symptomatically. Hence, new strategies are required to deepen the understanding of the disease and development of therapeutic strategies. The advent of stem cell and organoid technology has enabled the development of human pluripotent stem cell lines and adequate differentiation protocols for developing both airways and lung organoids in different formats. These novel human-pluripotent-stem-cell-derived organoids have enabled relatively accurate disease modeling. Idiopathic pulmonary fibrosis is a fatal and debilitating disease that exhibits prototypical fibrotic features that may be, to some extent, extrapolated to other conditions. Thus, respiratory diseases such as cystic fibrosis, chronic obstructive pulmonary disease, or the one caused by SARS-CoV-2 may reflect some fibrotic aspects reminiscent of those present in idiopathic pulmonary fibrosis. Modeling of fibrosis of the airways and the lung is a real challenge due to the large number of epithelial cells involved and interaction with other cell types of mesenchymal origin. This review will focus on the status of respiratory disease modeling from human-pluripotent-stem-cell-derived organoids, which are being used to model several representative respiratory diseases, such as idiopathic pulmonary fibrosis, cystic fibrosis, chronic obstructive pulmonary disease, and COVID-19.
Palabras clave
Pluripotent stem cells | Minilungs | Disease modeling | Fibrosis | Myofibroblasts | Idiopathic pulmonary fibrosis | IPF | Cystic fibrosis | CF | Chronic obstructive pulmonary disease | COPD | SARS-CoV-2 | COVID-19
MESH
COVID-19 | Cystic Fibrosis | Pluripotent Stem Cells | Pulmonary Disease, Chronic Obstructive | Respiration Disorders | Idiopathic Pulmonary Fibrosis | Humans | SARS-CoV-2 | Fibrosis | Organoids
Versión en línea
DOI
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