Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model.

ABSTRACT: Hepatobiliary disease causes significant morbidity in people with cystic fibrosis (CF), yet this problem remains understudied. We previously found that newborn CF pigs have microgallbladders with significant luminal obstruction in the absence of infection and consistent inflammation. In this study, we sought to better understand the early pathogenesis of CF pig gallbladder disease. We hypothesized that loss of CFTR would impair gallbladder epithelium anion/liquid secretion and increase mucin production. CFTR was expressed apically in non-CF pig gallbladder epithelium but was absent in CF. CF pig gallbladders lacked cAMP-stimulated anion transport. Using a novel gallbladder epithelial organoid model, we found that Cl^- or HCO₃^- was sufficient for non-CF organ

SUBMITTER: Zarei K 

PROVIDER: S-EPMC7578062 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature