{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T22:45:09Z","timestamp":1789771509225,"version":"4.0.1"},"reference-count":0,"publisher":"The Company of Biologists","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"am","delay-in-days":503,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"JSPS","award":["24228005"],"award-info":[{"award-number":["24228005"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Biliary atresia (BA) is characterized by the inflammation and obstruction of the extrahepatic bile ducts (EHBDs) in newborn infants. SOX17 is a master regulator of the fetal EHBDs formation. In mouse Sox17+\/\u2212 BA models, SOX17 reduction causes cell-autonomous epithelial shedding together with the ectopic appearance of SOX9-positive cystic duct-like epithelia in the gallbladder walls, resulting in the BA-like symptoms during the perinatal period. However, the similarities with the human BA gallbladders are still unclear. In the present study, we conducted the phenotypic analysis with the Sox17+\/\u2212 BA neonate mice, in order to compare with the gallbladder wall phenotype of human BA infants. The most characteristic phenotype of the Sox17+\/\u2212 BA gallbladders is the ectopic appearance of SOX9-positive peribiliary glands (PBGs), so-called pseudopyloric glands (PPGs). Next we examined SOX17\/SOX9 expression profiles of human gallbladders in thirteen BA infants. Among them, five BA cases showed a loss or drastic reduction of SOX17-positive signals throughout the whole region of gallbladder epithelia (SOX17-low group). Even in the remaining eight gallbladders (SOX17-high group), the epithelial cells near the decidual sites were frequently reduced in the SOX17-positive signal intensity. Most interestingly, the most characteristic phenotype of human BA gallbladders is the increased density of PBG\/PPG-like glands in the gallbladder body, especially near the epithelial decidual site, indicating the PBG\/PPG formation as a common phenotype between human BA and mouse Sox17+\/\u2212 BA gallbladders. These findings provide the first evidence of the potential contribution of SOX17 reduction and PBG\/PPG formation to the early pathogenesis in human BA gallbladders.<\/jats:p>","DOI":"10.1242\/dmm.042119","type":"journal-article","created":{"date-parts":[[2020,1,29]],"date-time":"2020-01-29T14:35:14Z","timestamp":1580308514000},"source":"Crossref","is-referenced-by-count":18,"title":["Gallbladder wall abnormality in biliary atresia of mouse <i>Sox17<\/i>+\/\u2212 neonates and human infants"],"prefix":"10.1242","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0036-3103","authenticated-orcid":false,"given":"Mami","family":"Uemura","sequence":"first","affiliation":[{"name":"Department of Veterinary Anatomy, the University of Tokyo, Tokyo 113-8657, Japan"},{"name":"Center for Experimental Animals, Tokyo Medical and Dental University, Tokyo 113-8510, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mayumi","family":"Higashi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Montri","family":"Pattarapanawan","sequence":"additional","affiliation":[{"name":"Department of Veterinary Anatomy, the University of Tokyo, Tokyo 113-8657, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shohei","family":"Takami","sequence":"additional","affiliation":[{"name":"Department of Veterinary Anatomy, the University of Tokyo, Tokyo 113-8657, Japan"},{"name":"Department of Pediatric Surgery, the University of Tokyo, Tokyo 113-0033, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Ichikawa","sequence":"additional","affiliation":[{"name":"Department of Veterinary Anatomy, the University of Tokyo, Tokyo 113-8657, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1324-8139","authenticated-orcid":false,"given":"Hiroki","family":"Higashiyama","sequence":"additional","affiliation":[{"name":"Department of Veterinary Anatomy, the University of Tokyo, Tokyo 113-8657, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taizo","family":"Furukawa","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Fujishiro","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, the University of Tokyo, Tokyo 113-0033, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuki","family":"Fukumura","sequence":"additional","affiliation":[{"name":"Department of Human Pathology, Juntendo University, Tokyo 113-8421, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Yao","sequence":"additional","affiliation":[{"name":"Department of Human Pathology, Juntendo University, Tokyo 113-8421, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuro","family":"Tajiri","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masami","family":"Kanai-Azuma","sequence":"additional","affiliation":[{"name":"Center for Experimental Animals, Tokyo Medical and Dental University, Tokyo 113-8510, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2116-7806","authenticated-orcid":false,"given":"Yoshiakira","family":"Kanai","sequence":"additional","affiliation":[{"name":"Department of Veterinary Anatomy, the University of Tokyo, Tokyo 113-8657, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2020,1,1]]},"container-title":["Disease Models &amp; Mechanisms"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1242\/dmm.042119","content-type":"unspecified","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.042119\/2065714\/dmm_042119v1.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"http:\/\/journals.biologists.com\/dmm\/article-pdf\/doi\/10.1242\/dmm.042119\/2065714\/dmm_042119v1.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T22:44:33Z","timestamp":1621377873000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/dmm\/article\/doi\/10.1242\/dmm.042119\/267151\/Gallbladder-wall-abnormality-in-biliary-atresia-of"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,1,1]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1242\/dmm.042119","relation":{},"ISSN":["1754-8411","1754-8403"],"issn-type":[{"value":"1754-8411","type":"electronic"},{"value":"1754-8403","type":"print"}],"subject":[],"published":{"date-parts":[[2020,1,1]]},"article-number":"dmm.042119"}}