{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Zarei K"],"funding":["U.S. Department of Health &amp; Human Services | NIH | National Institute of General Medical Sciences","NIDDK NIH HHS","NIEHS NIH HHS","Cystic Fibrosis Foundation","NHLBI NIH HHS","U.S. Department of Health &amp; Human Services | NIH | National Heart, Lung, and Blood Institute","NIGMS NIH HHS"],"pagination":["1388-1399"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC7578062"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["100(11)"],"pubmed_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<sup>-</sup> or HCO<sub>3</sub><sup>-</sup> was sufficient for non-CF organ"],"journal":["Laboratory investigation; a journal of technical methods and pathology"],"pubmed_title":["Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model."],"pmcid":["PMC7578062"],"funding_grant_id":["K01 HL140261","T32 HL007638","HL091842","P30 DK054759","P01 HL091842","P30 ES005605","STOLTZ19R0","T32 GM007337","P01 HL051670","GM007337","HL007638","R01 HL136813"],"pubmed_authors":["Stroik MR","Meyerholz DK","Zarei K","Ernst SE","Powers LS","Gansemer ND","Thornell IM","Pezzulo AA","Stoltz DA","Thurman AL","Ostedgaard LS"],"additional_accession":[]},"is_claimable":false,"name":"Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model.","description":"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<sup>-</sup> or HCO<sub>3</sub><sup>-</sup> was sufficient for non-CF organ","dates":{"release":"2020-01-01T00:00:00Z","publication":"2020 Nov","modification":"2026-05-01T01:29:41.283Z","creation":"2021-02-21T03:42:01Z"},"accession":"S-EPMC7578062","cross_references":{"pubmed":["32719544"],"doi":["10.1038/s41374-020-0474-8"]}}