{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T03:19:23Z","timestamp":1790133563854,"version":"4.0.1"},"reference-count":54,"publisher":"The Company of Biologists","issue":"22","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2004,11,15]]},"abstract":"<jats:p>The Alagille Syndrome (AGS) is a heritable disorder affecting the liver and other organs. Causative dominant mutations in human Jagged 1 have been identified in most AGS patients. Related organ defects occur in mice that carry jagged 1 and notch 2 mutations. Multiple jagged and notch genes are expressed in the developing zebrafish liver. Compound jagged and notch gene knockdowns alter zebrafish biliary, kidney, pancreatic, cardiac and craniofacial development in a manner compatible with an AGS phenocopy. These data confirm an evolutionarily conserved role for Notch signaling in vertebrate liver development, and support the zebrafish as a model system for diseases of the human biliary system.<\/jats:p>","DOI":"10.1242\/dev.01411","type":"journal-article","created":{"date-parts":[[2004,10,27]],"date-time":"2004-10-27T21:58:17Z","timestamp":1098914297000},"page":"5753-5766","source":"Crossref","is-referenced-by-count":197,"title":["Inhibition of Jagged-mediated Notch signaling disrupts zebrafish biliary development and generates multi-organ defects compatible with an Alagille syndrome phenocopy"],"prefix":"10.1242","volume":"131","author":[{"given":"Kristin","family":"Lorent","sequence":"first","affiliation":[{"name":"Department of Medicine, University of Pennsylvania School of Medicine, 421 Curie Boulevard, Philadelphia, PA 19104-6058, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sang-Yeob","family":"Yeo","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Genetics, NICHD, NIH, 31 Center Drive, 9000 Rockville Pike, Bethesda, MD 20892-2425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takaya","family":"Oda","sequence":"additional","affiliation":[{"name":"Genome Technology Branch, NHGRI, NIH, 49 Convent Drive, 9000 Rockville Pike,Bethesda, MD 20892-2152, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Settara","family":"Chandrasekharappa","sequence":"additional","affiliation":[{"name":"Genome Technology Branch, NHGRI, NIH, 49 Convent Drive, 9000 Rockville Pike,Bethesda, MD 20892-2152, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ajay","family":"Chitnis","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular Genetics, NICHD, NIH, 31 Center Drive, 9000 Rockville Pike, Bethesda, MD 20892-2425, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Randolph P.","family":"Matthews","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Nutrition, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Pack","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Pennsylvania School of Medicine, 421 Curie Boulevard, Philadelphia, PA 19104-6058, USA"},{"name":"Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-6058, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","reference":[{"key":"2021042612051490200_REF1","doi-asserted-by":"crossref","unstructured":"Apelqvist, A., Li, H., Sommer, L., Beatus, P., Anderson, D. 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