ArticleJournal of hepatology2020
Coordinated development of the mouse extrahepatic bile duct: Implications for neonatal susceptibility to biliary injury.
Article in Journal of hepatology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
What it found
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Who cites it
13 citing papers in PubMed, 33 citations in OpenAlex.
- Understanding sub-cellular mechanochemical correlations of the neonatal extrahepatic bile duct extracellular matrix by atomic force microscope-Infrared spectroscopy.Analytica chimica acta · 2026Article
- Biliary atresia susceptibility gene EFEMP1 regulates extrahepatic bile duct elastic fiber formation and mechanics.JHEP reports : innovation in hepatology · 2025Article
- Genetic background and biliary atresia.World journal of pediatric surgery · 2025Review
- Biliary atresia.Nature reviews. Disease primers · 2024Review
- A fetal wound healing program after intrauterine bile duct injury may contribute to biliary atresia.Journal of hepatology · 2023Article
- Contribution ofInternational journal of molecular sciences · 2023Article
- Biliatresone induces cholangiopathy in C57BL/6J neonates.Scientific reports · 2023Article
- Development of extrahepatic bile ducts and mechanisms of tumorigenesis: Lessons from mouse models.Pathology international · 2022Review
- Bile acid-sensitive tuft cells regulate biliary neutrophil influx.Science immunology · 2022Article
- Rappaport, Glisson, Hering, and Mall-Champions of Liver Microanatomy: Microscopic and Ultramicroscopic Anatomy of the Liver Into the Modern Age.Clinical liver disease · 2021Review
- Evidence for continuity of interstitial spaces across tissue and organ boundaries in humans.Communications biology · 2021Article
- Congenital biliary atresia is correlated with disrupted cell junctions and polarity caused by Cdc42 insufficiency in the liver.Theranostics · 2021Article
- In Utero Extrahepatic Bile Duct Damage and Repair: Implications for Biliary Atresia.Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyReview
Corrections and comments
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Authors and funding
12 authors at 4 institutions in 2 countries.
Funding
Abstract
BACKGROUND &
aimsThe extrahepatic bile duct is the primary tissue initially affected by biliary atresia. Biliary atresia is a cholangiopathy which exclusively affects neonates. Current animal models suggest that the developing bile duct is uniquely susceptible to damage. In this study, we aimed to define the anatomical and functional differences between the neonatal and adult mouse extrahepatic bile ducts.
methodsWe studied mouse passaged cholangiocytes, mouse BALB/c neonatal and adult primary cholangiocytes, as well as isolated extrahepatic bile ducts, and a collagen reporter mouse. The methods used included transmission electron microscopy, lectin staining, immunostaining, rhodamine uptake assays, bile acid toxicity assays, and in vitro modeling of the matrix.
resultsThe cholangiocyte monolayer of the neonatal extrahepatic bile duct was immature, lacking the uniform apical glycocalyx and mature cell-cell junctions typical of adult cholangiocytes. Functional studies showed that the glycocalyx protected against bile acid injury and that neonatal cholangiocyte monolayers were more permeable than adult monolayers. In adult ducts, the submucosal space was filled with collagen I, elastin, hyaluronic acid, and proteoglycans. In contrast, the neonatal submucosa had little collagen I and elastin, although both increased rapidly after birth. In vitro modeling of the matrix suggested that the composition of the neonatal submucosa relative to the adult submucosa led to increased diffusion of bile. A Col-GFP reporter mouse showed that cells in the neonatal but not adult submucosa were actively producing collagen.
conclusionWe identified 4 key differences between the neonatal and adult extrahepatic bile duct. We showed that these features may have functional implications, suggesting the neonatal extrahepatic bile ducts are particularly susceptible to injury and fibrosis. LAY SUMMARY: Biliary atresia is a disease that affects newborns and is characterized by extrahepatic bile duct injury and obstruction, resulting in liver injury. We identify 4 key differences between the epithelial and submucosal layers of the neonatal and adult extrahepatic bile duct and show that these may render the neonatal duct particularly susceptible to injury.
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