{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:21:39Z","timestamp":1787008899483,"version":"build-2736575974"},"reference-count":33,"publisher":"Oxford University Press (OUP)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,3,15]]},"DOI":"10.1093\/hmg\/ddr575","type":"journal-article","created":{"date-parts":[[2011,12,8]],"date-time":"2011-12-08T23:31:53Z","timestamp":1323387113000},"page":"1374-1383","source":"Crossref","is-referenced-by-count":65,"title":["Cranial neural crest ablation of Jagged1 recapitulates the craniofacial phenotype of Alagille syndrome patients"],"prefix":"10.1093","volume":"21","author":[{"given":"Ryan","family":"Humphreys","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lawrence S","family":"Prince","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xianghu","family":"Qu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Brown","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen","family":"Loomes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stacey S.","family":"Huppert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Baldwin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Goudy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2011,12,8]]},"reference":[{"key":"key\n\t\t\t\t20170522143758_DDR575C1","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/S0022-3476(75)80706-2","article-title":"Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur","volume":"86","author":"Alagille","year":"1975","journal-title":"J. Pediatr."},{"key":"key\n\t\t\t\t20170522143758_DDR575C2","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1038\/ng0797-235","article-title":"Mutations in the human Jagged1 gene are responsible for Alagille syndrome","volume":"16","author":"Oda","year":"1997","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C3","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1038\/ng0797-243","article-title":"Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1","volume":"16","author":"Li","year":"1997","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C4","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1002\/hep.510290331","article-title":"Features of Alagille syndrome in 92 patients: frequency and relation to prognosis","volume":"29","author":"Emerick","year":"1999","journal-title":"Hepatology"},{"key":"key\n\t\t\t\t20170522143758_DDR575C5","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1055\/s-2001-19036","article-title":"Alagille syndrome and the Jagged1 gene","volume":"21","author":"Piccoli","year":"2001","journal-title":"Semin. Liver Dis."},{"key":"key\n\t\t\t\t20170522143758_DDR575C6","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/S0022-3476(95)70298-9","article-title":"Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy","volume":"127","author":"Hoffenberg","year":"1995","journal-title":"J. Pediatr."},{"key":"key\n\t\t\t\t20170522143758_DDR575C7","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1002\/ajmg.1320350419","article-title":"Alagille syndrome (arteriohepatic dysplasia) and del(20)(p11.2)","volume":"35","author":"Legius","year":"1990","journal-title":"Am. J. Med. Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C8","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1002\/ajmg.10579","article-title":"Facial features in Alagille syndrome: specific or cholestasis facies?","volume":"112","author":"Kamath","year":"2002","journal-title":"Am. J. Med. Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C9","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1016\/j.ydbio.2006.07.042","article-title":"Molecular control of secondary palate development","volume":"301","author":"Gritli-Linde","year":"2007","journal-title":"Dev. Biol."},{"key":"key\n\t\t\t\t20170522143758_DDR575C10","doi-asserted-by":"crossref","first-page":"1843","DOI":"10.1242\/dev.049056","article-title":"Jagged-Notch signaling ensures dorsal skeletal identity in the vertebrate face","volume":"137","author":"Zuniga","year":"2010","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522143758_DDR575C11","doi-asserted-by":"crossref","first-page":"1671","DOI":"10.1242\/dev.127.8.1671","article-title":"Fate of the mammalian cranial neural crest during tooth and mandibular morphogenesis","volume":"127","author":"Chai","year":"2000","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522143758_DDR575C12","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1016\/S0960-9822(07)00562-3","article-title":"Modification of gene activity in mouse embryos in utero by a tamoxifen-inducible form of Cre recombinase","volume":"8","author":"Danielian","year":"1998","journal-title":"Curr. Biol."},{"key":"key\n\t\t\t\t20170522143758_DDR575C13","first-page":"1986","article-title":"Murine Jagged1\/Notch signaling in the second heart field orchestrates Fgf8 expression and tissue-tissue interactions during outflow tract development","volume":"119","author":"High","year":"2009","journal-title":"J. Clin. Invest."},{"key":"key\n\t\t\t\t20170522143758_DDR575C14","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1002\/ajmg.10175","article-title":"Craniofacial phenotypes in segmentally trisomic mouse models for Down syndrome","volume":"107","author":"Richtsmeier","year":"2002","journal-title":"Am. J. Med. Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C15","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1093\/hmg\/8.5.723","article-title":"Embryonic lethality and vascular defects in mice lacking the Notch ligand Jagged1","volume":"8","author":"Xue","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C16","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1016\/j.ydbio.2010.02.021","article-title":"Neural crest cell-specific deletion of Rac1 results in defective cell-matrix interactions and severe craniofacial and cardiovascular malformations","volume":"340","author":"Thomas","year":"2010","journal-title":"Dev. Biol."},{"key":"key\n\t\t\t\t20170522143758_DDR575C17","doi-asserted-by":"crossref","first-page":"20349","DOI":"10.1074\/jbc.M500273200","article-title":"The extracellular matrix protein MAGP-2 interacts with Jagged1 and induces its shedding from the cell surface","volume":"280","author":"Nehring","year":"2005","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522143758_DDR575C18","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1002\/bdrc.20171","article-title":"Extracellular matrix provides an optimal niche for the maintenance and propagation of mesenchymal stem cells","volume":"90","author":"Chen","year":"2010","journal-title":"Birth Defects Res. C. Embryo Today"},{"key":"key\n\t\t\t\t20170522143758_DDR575C19","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1016\/j.mod.2010.03.001","article-title":"Tbx1 is necessary for palatal elongation and elevation","volume":"127","author":"Goudy","year":"2010","journal-title":"Mech. Dev."},{"key":"key\n\t\t\t\t20170522143758_DDR575C20","doi-asserted-by":"crossref","first-page":"2515","DOI":"10.1073\/pnas.0913985107","article-title":"Analysis of a gain-of-function FGFR2 Crouzon mutation provides evidence of loss of function activity in the etiology of cleft palate","volume":"107","author":"Snyder-Warwick","year":"2010","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522143758_DDR575C21","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1242\/dev.01074","article-title":"Notch signaling: control of cell communication and cell fate","volume":"131","author":"Lai","year":"2004","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522143758_DDR575C22","doi-asserted-by":"crossref","first-page":"1461","DOI":"10.1242\/dev.042911","article-title":"RBPjkappa-dependent Notch signaling regulates mesenchymal progenitor cell proliferation and differentiation during skeletal development","volume":"137","author":"Dong","year":"0000","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522143758_DDR575C23","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1002\/dvdy.20821","article-title":"Jag2-Notch1 signaling regulates oral epithelial differentiation and palate development","volume":"235","author":"Casey","year":"2006","journal-title":"Dev. Dyn."},{"key":"key\n\t\t\t\t20170522143758_DDR575C24","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1242\/dev.129.4.1075","article-title":"A mouse model of Alagille syndrome: Notch2 as a genetic modifier of Jag1 haploinsufficiency","volume":"129","author":"McCright","year":"2002","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522143758_DDR575C25","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1002\/gene.10241","article-title":"Characterization of Notch3-deficient mice: normal embryonic development and absence of genetic interactions with a Notch1 mutation","volume":"37","author":"Krebs","year":"2003","journal-title":"Genesis"},{"key":"key\n\t\t\t\t20170522143758_DDR575C26","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1101\/gad.14.11.1343","article-title":"Notch signaling is essential for vascular morphogenesis in mice","volume":"14","author":"Krebs","year":"2000","journal-title":"Genes Dev."},{"key":"key\n\t\t\t\t20170522143758_DDR575C27","doi-asserted-by":"crossref","first-page":"3555","DOI":"10.1242\/dev.022319","article-title":"Notch signaling is required for the maintenance of enteric neural crest progenitors","volume":"135","author":"Okamura","year":"2008","journal-title":"Development"},{"key":"key\n\t\t\t\t20170522143758_DDR575C28","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1161\/01.ATV.0000060892.81529.8F","article-title":"Notch signaling in vascular development","volume":"23","author":"Iso","year":"2003","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"key\n\t\t\t\t20170522143758_DDR575C29","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/j.mod.2008.06.007","article-title":"Cell lineage in mammalian craniofacial mesenchyme","volume":"125","author":"Yoshida","year":"2008","journal-title":"Mech. Dev."},{"key":"key\n\t\t\t\t20170522143758_DDR575C30","doi-asserted-by":"crossref","first-page":"1955","DOI":"10.1073\/pnas.0709663105","article-title":"Endothelial expression of the Notch ligand Jagged1 is required for vascular smooth muscle development","volume":"105","author":"High","year":"2008","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522143758_DDR575C31","doi-asserted-by":"crossref","first-page":"e4","DOI":"10.1371\/journal.pgen.0020004","article-title":"The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear","volume":"2","author":"Kiernan","year":"2006","journal-title":"PLoS Genet."},{"key":"key\n\t\t\t\t20170522143758_DDR575C32","doi-asserted-by":"crossref","first-page":"1692","DOI":"10.1172\/JCI20384","article-title":"Disruption of Fgf10\/Fgfr2b-coordinated epithelial-mesenchymal interactions causes cleft palate","volume":"113","author":"Rice","year":"2004","journal-title":"J. Clin. Invest."},{"key":"key\n\t\t\t\t20170522143758_DDR575C33","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.1002\/hep.23431","article-title":"Notch signaling regulates formation of the three-dimensional architecture of intrahepatic bile ducts in mice","volume":"51","author":"Sparks","year":"0000","journal-title":"Hepatology"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/21\/6\/1374\/17258723\/ddr575.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,19]],"date-time":"2021-12-19T08:53:33Z","timestamp":1639904013000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddr575"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,12,8]]},"references-count":33,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2011,12,8]]},"published-print":{"date-parts":[[2012,3,15]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddr575","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2011,12,8]]}}}