{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T06:18:55Z","timestamp":1792736335007,"version":"4.5.0"},"reference-count":28,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2013,7,1]],"date-time":"2013-07-01T00:00:00Z","timestamp":1372636800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2013,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objectives:<\/jats:title><jats:p>Biliary atresia (BA) is a rapidly progressive form of biliary fibrosis affecting neonates. We previously reported that primary cilia on the intrahepatic cholangiocytes of patients with both syndromic and nonsyndromic BA were structurally abnormal. Our objective was to determine whether extrahepatic cholangiocytes in human biliary atesia, intrahepatic and extrahepatic cholangiocytes of rhesus rotavirus (RRV)\u2010infected neonatal mice, and RRV\u2010infected primary neonatal extrahepatic cholangiocytes also demonstrate ciliary abnormalities.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p>The livers of neonatal BALB\/c mice injected with RRV that developed jaundice, human extrahepatic bile duct samples obtained at time of hepatoportoenterostomy, and RRV\u2010infected primary neonatal cholangiocytes were stained with antibodies against acetylated \u03b1 tubulin to identify primary cilia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Extrahepatic cholangiocytes from RRV\u2010treated mice demonstrated minimal loss of primary cilia at day 3 but almost complete loss at day 8 and partial loss at day 12. No changes were seen in mouse intrahepatic bile ducts at any of the time points. In the human BA samples, primary cilia were almost completely absent from extrahepatic duct cholangiocytes. There were, however, abundant cilia in the peribiliary glands adjacent to extrahepatic ducts in the BA sample. Cilia in RRV\u2010infected primary neonatal cholangiocytes were significantly decreased compared with controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>Primary cilia are selectively lost from neonatal extrahepatic but not intrahepatic cholangiocytes after RRV infection in BALB\/c mice. The cilia are also decreased in RRV\u2010infected primary cholangiocytes and the extrahepatic ducts from human patients with BA. This suggests that ciliary abnormalities are part of the pathophysiology of BA.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e318296e525","type":"journal-article","created":{"date-parts":[[2013,4,19]],"date-time":"2013-04-19T14:23:00Z","timestamp":1366381380000},"page":"96-101","source":"Crossref","is-referenced-by-count":41,"title":["Extrahepatic Cholangiocyte Cilia Are Abnormal in Biliary Atresia"],"prefix":"10.1002","volume":"57","author":[{"given":"Sara","family":"Karjoo","sequence":"first","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition The Children's Hospital of Philadelphia Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas J.","family":"Hand","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition The Children's Hospital of Philadelphia Philadelphia 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