Open AccessStem cell‐derived organoids to model gastrointestinal facets of cystic fibrosis
Author(s) -
Hohwieler Meike,
Perkhofer Lukas,
Liebau Stefan,
Seufferlein Thomas,
Müller Martin,
Illing Anett,
Kleger Alexander
Publication year - 2017
Publication title -
ueg journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.667
H-Index - 35
eISSN - 2050-6414
pISSN - 2050-6406
DOI - 10.1177/2050640616670565
Subject(s) - medicine , organoid , cystic fibrosis , stem cell , pathology , microbiology and biotechnology , biology
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.