ArticleAmerican journal of physiology. Gastrointestinal and liver physiology2024
Acquired dysfunction of CFTR underlies cystic fibrosis-like disease of the canine gallbladder.
Article in American journal of physiology. Gastrointestinal and liver physiology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Histological, ultrastructural, and single-cell profiling reveal immune-mediated remodeling in gallbladder inflammation.Cell and tissue research · 2026Article
- Gallbladder mucoceles in dogs: a novel form of acquired CFTR dysfunction causing localized cystic fibrosis-like disease.American journal of physiology. Gastrointestinal and liver physiology · 2024Article
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11 authors.
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Abstract
Mucocele formation in dogs is a unique and enigmatic muco-obstructive disease of the gallbladder caused by the amassment of abnormal mucus that bears striking pathological similarity to cystic fibrosis. We investigated the role of cystic fibrosis transmembrane conductance regulatory protein (CFTR) in the pathogenesis of this disease. The location and frequency of disease-associated variants in the coding region of CFTR were compared using whole genome sequence data from 2,642 dogs representing breeds at low-risk, high-risk, or with confirmed disease. Expression, localization, and ion transport activity of CFTR were quantified in control and mucocele gallbladders by NanoString, Western blotting, immunofluorescence imaging, and studies in Ussing chambers. Our results establish a significant loss of CFTR-dependent anion secretion by mucocele gallbladder mucosa. A significantly lower quantity of CFTR protein was demonstrated relative to E-cadherin in mucocele compared with control gallbladder mucosa. Immunofluorescence identified CFTR along the apical membrane of epithelial cells in control gallbladders but not in mucocele gallbladder epithelium. Decreases in mRNA copy number for
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