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Theratyping cystic fibrosis in vitro in ALI culture and organoid models generated from patient-derived nasal epithelial conditionally reprogrammed stem cells

QUESTION: Cystic fibrosis (CF) is due to pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Recent improvements have enabled pharmacological therapy aiming at restoring mutated CFTR expression and function. CFTR “modulators” have revolutionised the CF therape...

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Detalles Bibliográficos
Autores principales: Sette, Giovanni, Lo Cicero, Stefania, Blaconà, Giovanna, Pierandrei, Silvia, Bruno, Sabina Maria, Salvati, Valentina, Castelli, Germana, Falchi, Mario, Fabrizzi, Benedetta, Cimino, Giuseppe, De Maria, Ruggero, Biffoni, Mauro, Eramo, Adriana, Lucarelli, Marco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8675295/
https://www.ncbi.nlm.nih.gov/pubmed/34413153
http://dx.doi.org/10.1183/13993003.00908-2021