A functional CFTR assay using primary cystic fibrosis intestinal organoids

• Published in: Nature medicine Vol. 19; no. 7; pp. 939 - 945
• Main Authors: Dekkers, Johanna F, Wiegerinck, Caroline L, de Jonge, Hugo R, Bronsveld, Inez, Janssens, Hettie M, de Winter-de Groot, Karin M, Brandsma, Arianne M, de Jong, Nienke W M, Bijvelds, Marcel J C, Scholte, Bob J, Nieuwenhuis, Edward E S, van den Brink, Stieneke, Clevers, Hans, van der Ent, Cornelis K, Middendorp, Sabine, Beekman, Jeffrey M
• Format: Journal Article
• Language: English
• Published: New York Nature Publishing Group US 01.07.2013; Nature Publishing Group
• Subjects: 1678/631/45/269; 1877/631/532/2437; 347/631/1647/1407/652; 600/631/1647/767/2199; Animals; Biological Assay - methods; Biomedicine; Biotechnology; Cancer Research; Cells, Cultured; Cystic fibrosis; Cystic Fibrosis - diagnosis; Cystic Fibrosis - genetics; Cystic Fibrosis - pathology; Cystic Fibrosis Transmembrane Conductance Regulator - analysis; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Drug therapy; Female; HEK293 Cells; Humans; Incubation; Infectious Diseases; Intestinal Mucosa - metabolism; Intestinal Mucosa - pathology; Low temperature; Male; Metabolic Diseases; Mice; Mice, Inbred C57BL; Mice, Inbred CFTR; Molecular Medicine; Mutagenesis; Mutant Proteins - analysis; Mutant Proteins - genetics; Mutant Proteins - metabolism; Mutant Proteins - physiology; Mutation; Neurosciences; Organoids - metabolism; Organoids - pathology; Primary Cell Culture - methods; technical-report; Technology application; Tissues; Water - metabolism; Netherlands

Building on earlier work, Dekkers et al . describe the first application of their intestinal organoid culture technology to the study of human disease, in this case cystic fibrosis. These so called 'mini-guts', which recapitulate the essential in vivo intestinal tissue architecture in vitr...