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https://hdl.handle.net/1822/62010
Título: | A three-dimensional model of human lung development and disease from pluripotent stem cells |
Autor(es): | Chen, Ya-Wen Huang, Sarah Xuelian de Carvalho, Ana Luisa Rodrigues Toste Ho, Siu-Hong Islam, Mohammad Naimul Volpi, Stefano Notarangelo, Luigi D. Ciancanelli, Michael Casanova, Jean-Laurent Bhattacharya, Jahar Liang, Alice F. Palermo, Laura M. Porotto, Matteo Moscona, Anne Snoeck, Hans-Willem |
Palavras-chave: | Animals Cell Differentiation Cells, Cultured Female Genetic Predisposition to Disease Humans Lung Lung Transplantation Male Membrane Proteins Mice, Inbred NOD Mutation Organogenesis Organoids Phenotype Pluripotent Stem Cells Pulmonary Fibrosis Respiratory Syncytial Virus Infections Time Factors Tissue Engineering Cell Culture Techniques |
Data: | Mai-2017 |
Editora: | Nature Research |
Revista: | Nature Cell Biology |
Citação: | Chen, Y. W., Huang, S. X., De Carvalho, A. L. R. T., Ho, S. H., Islam, M. N., Volpi, S., ... & Liang, A. F. (2017). A three-dimensional model of human lung development and disease from pluripotent stem cells. Nature cell biology, 19(5), 542. |
Resumo(s): | Recapitulation of lung development from human pluripotent stem cells (hPSCs) in three dimensions (3D) would allow deeper insight into human development, as well as the development of innovative strategies for disease modelling, drug discovery and regenerative medicine. We report here the generation from hPSCs of lung bud organoids (LBOs) that contain mesoderm and pulmonary endoderm and develop into branching airway and early alveolar structures after xenotransplantation and in Matrigel 3D culture. Expression analysis and structural features indicated that the branching structures reached the second trimester of human gestation. Infection in vitro with respiratory syncytial virus, which causes small airway obstruction and bronchiolitis in infants, led to swelling, detachment and shedding of infected cells into the organoid lumens, similar to what has been observed in human lungs. Introduction of mutation in HPS1, which causes an early-onset form of intractable pulmonary fibrosis, led to accumulation of extracellular matrix and mesenchymal cells, suggesting the potential use of this model to recapitulate fibrotic lung disease in vitro. LBOs therefore recapitulate lung development and may provide a useful tool to model lung disease. |
Tipo: | Artigo |
Descrição: | Author Manuscript |
URI: | https://hdl.handle.net/1822/62010 |
DOI: | 10.1038/ncb3510 |
ISSN: | 1465-7392 |
e-ISSN: | 1476-4679 |
Versão da editora: | https://www.nature.com/articles/ncb3510 |
Arbitragem científica: | yes |
Acesso: | Acesso restrito UMinho |
Aparece nas coleções: | ICVS - Artigos em revistas internacionais / Papers in international journals |
Ficheiros deste registo:
Ficheiro | Descrição | Tamanho | Formato | |
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Chen-2017-A-three-dimensional-model-of-human-.pdf Acesso restrito! | 3,99 MB | Adobe PDF | Ver/Abrir |