Back to Search Start Over

Stem cell‐derived organoids to model gastrointestinal facets of cystic fibrosis

Authors :
Lukas Perkhofer
Stefan Liebau
Meike Hohwieler
Thomas Seufferlein
Anett Illing
Martin Müller
Alexander Kleger
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.

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