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Stem cell‐derived organoids to model gastrointestinal facets of cystic fibrosis
- Source :
- United European Gastroenterology Journal. 5:609-624
- Publication Year :
- 2017
- Publisher :
- Wiley, 2017.
-
Abstract
- Cystic fibrosis (CF) is one of the most frequently occurring inherited human diseases caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) which lead to ample defects in anion transport and epithelial fluid secretion. Existing models lack both access to early stages of CF development and a coeval focus on the gastrointestinal CF phenotypes, which become increasingly important due increased life span of the affected individuals. Here, we provide a comprehensive overview of gastrointestinal facets of CF and the opportunity to model these in various systems in an attempt to understand and treat CF. A particular focus is given on forward-leading organoid cultures, which may circumvent current limitations of existing models and thereby provide a platform for drug testing and understanding of disease pathophysiology in gastrointestinal organs.
- Subjects :
- 0301 basic medicine
biology
business.industry
Gastroenterology
Review Article
Disease
medicine.disease
Cystic fibrosis
Phenotype
Cystic fibrosis transmembrane conductance regulator
03 medical and health sciences
030104 developmental biology
0302 clinical medicine
Directed differentiation
Oncology
030220 oncology & carcinogenesis
Immunology
biology.protein
medicine
Organoid
Stem cell
Induced pluripotent stem cell
business
Subjects
Details
- ISSN :
- 20506414 and 20506406
- Volume :
- 5
- Database :
- OpenAIRE
- Journal :
- United European Gastroenterology Journal
- Accession number :
- edsair.doi.dedup.....c236fb006ae7a4ffb7d0d37cc16a4c5d
- Full Text :
- https://doi.org/10.1177/2050640616670565