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Biochim Biophys Acta Mol Basis Dis. 2019 May 1;1865(5):965-969. doi: 10.1016/j.bbadis.2018.07.026. Epub 2018 Jul 30.

Animal models for cystic fibrosis liver disease (CFLD).

Author information

1
Digestive Disease Section, Yale Liver Center, Yale University School of Medicine, New Haven, CT, USA.
2
Department of Molecular Medicine, University of Padova School of Medicine, Padova, Italy.
3
Digestive Disease Section, Yale Liver Center, Yale University School of Medicine, New Haven, CT, USA. Electronic address: carlo.spirli@yale.edu.

Abstract

Liver disease is a severe complication in patients with Cystic Fibrosis (CF), a genetic disease caused by mutations in the gene encoding for cystic fibrosis transmembrane conductance regulator (CFTR) channel. The sequence of events leading to CFLD is still unclear and has limited the development of more specific treatments other than the bile acid UDCA. However, in the last twenty years, several gaps have been filled, which have mainly been possible due to the availability of different animal models that mimic CF. CF mice, although they lack a spontaneous liver manifestation, have been essential to better understand the multiple functions of CFTR expression on the apical membrane of cholangiocytes, from chloride channel to regulator of epithelial innate immunity. Additionally, we have learned that the gut microbiota might be a pathogenetic factor for the development of liver disease. The recent creation of novel CF animal models (i.e. pig and ferret) that better reproduce the human disease, will allow for comparative studies with species that spontaneously develop the liver disease and will hopefully lead to novel therapeutic treatments. In this review, we have compared and summarized the main features of the current available CF animal models and their applicability for the study of the liver phenotype.

KEYWORDS:

Biliary secretion; CFTR; Cholangiocytes; Inflammation; Microbiota

PMID:
30071276
PMCID:
PMC6474816
[Available on 2020-05-01]
DOI:
10.1016/j.bbadis.2018.07.026

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