{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T11:24:58Z","timestamp":1769599498884,"version":"3.49.0"},"reference-count":25,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,3,1]],"date-time":"2011-03-01T00:00:00Z","timestamp":1298937600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K08 DK068009"],"award-info":[{"award-number":["K08 DK068009"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R03 DK080710"],"award-info":[{"award-number":["R03 DK080710"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2011,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background:<\/jats:title><jats:p><jats:italic>zfhz1b<\/jats:italic> is the causative gene for Mowat\u2010Wilson syndrome, in which patients demonstrate developmental delay and Hirschsprung disease, as well as other anomalies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods:<\/jats:title><jats:p>We identified a patient with Mowat\u2010Wilson syndrome who also developed cholestasis and histopathologic features consistent with biliary atresia, suggesting that mutations involving <jats:italic>zfhz1b<\/jats:italic> may lead to biliary developmental anomalies or injury to the biliary tract. We used the zebrafish model system to determine whether <jats:italic>zfhx1b<\/jats:italic> has a role in vertebrate biliary development.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Using zebrafish we determined that <jats:italic>zfhx1b<\/jats:italic> was expressed in the developing liver during biliary growth and remodeling, and that morpholino antisense oligonucleotide\u2013mediated knockdown of <jats:italic>zfhx1b<\/jats:italic> led to defects in biliary development. These findings were associated with decreased expression of <jats:italic>vhnf1<\/jats:italic>, a transcription factor known to be important in biliary development in zebrafish and in mammals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>Our studies underscore the importance of genetic contributions in the etiology of infantile hepatobiliary disorders, including biliary atresia.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e3181ff2e5b","type":"journal-article","created":{"date-parts":[[2011,2,17]],"date-time":"2011-02-17T08:15:35Z","timestamp":1297930535000},"page":"339-344","source":"Crossref","is-referenced-by-count":17,"title":["Intrahepatic Biliary Anomalies in a Patient With Mowat\u2010Wilson Syndrome Uncover a Role for the Zinc Finger Homeobox Gene <i>zfhx1b<\/i> in Vertebrate Biliary Development"],"prefix":"10.1002","volume":"52","author":[{"given":"Shuang","family":"Cui","sequence":"first","affiliation":[{"name":"The Children's Hospital of Philadelphia Research Institute USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jessi","family":"Erlichman","sequence":"additional","affiliation":[{"name":"The Children's Hospital of Philadelphia Research Institute USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierre","family":"Russo","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Pennsylvania School of Medicine Philadelphia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara A","family":"Haber","sequence":"additional","affiliation":[{"name":"The Children's Hospital of Philadelphia Research Institute USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Randolph P","family":"Matthews","sequence":"additional","affiliation":[{"name":"The Children's Hospital of Philadelphia Research Institute 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