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Phenotypes Associated with This Genotype
Genotype
MGI:5318528
Allelic
Composition
Jag1tm2Grid/Jag1tm2Grid
Tg(Wnt1-cre)11Rth/0
Genetic
Background
involves: 129S1/Sv * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Jag1tm2Grid mutation (1 available); any Jag1 mutation (8 available)
Tg(Wnt1-cre)11Rth mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• maxilla deficiencies lead to poor feeding and death around one month of age (J:181120)
• maxilla deficiencies lead to poor feeding and death around one month of age (J:181120)

growth/size/body
• the palatal dimensions are proportionally smaller, however the structural components are in tact (J:181120)
• mutants exhibit reduced proliferation in the palate shelves at E14.5 and in the adjacent palatal epithelium (J:181120)
• mutants exhibit reduced hyaluronic acid in the palate shelves at E14.5 (J:181120)
• mutants exhibit aberrant vascular pattering in the palate including reduced vascular branching at E14.5 and E15.5 (J:181120)
• the palatal dimensions are proportionally smaller, however the structural components are in tact (J:181120)
• mutants exhibit reduced proliferation in the palate shelves at E14.5 and in the adjacent palatal epithelium (J:181120)
• mutants exhibit reduced hyaluronic acid in the palate shelves at E14.5 (J:181120)
• mutants exhibit aberrant vascular pattering in the palate including reduced vascular branching at E14.5 and E15.5 (J:181120)
• at E14.5, palatal shelf elevation above the tongue occurs normally but palatal elongation is reduced anteriorly, whereas the posterior palate shelf length is unaffected (J:181120)
• mutants exhibit delayed palatal shelf elongation (J:181120)
• some E15.5 mutants show delayed palate shelf apposition and the persistence of the epithelial seam, however palate fusion occurs in all mutants (J:181120)
• at E14.5, palatal shelf elevation above the tongue occurs normally but palatal elongation is reduced anteriorly, whereas the posterior palate shelf length is unaffected (J:181120)
• mutants exhibit delayed palatal shelf elongation (J:181120)
• some E15.5 mutants show delayed palate shelf apposition and the persistence of the epithelial seam, however palate fusion occurs in all mutants (J:181120)
• palate shelf height is reduced in both the anterior and posterior regions (J:181120)
• palate shelf height is reduced in both the anterior and posterior regions (J:181120)
• midfacial hypoplasia resulting in a reduction in anterior facial width (J:181120)
• midfacial hypoplasia resulting in a reduction in anterior facial width (J:181120)

craniofacial
• shortened maxillary regions; anterior-posterior facial length is normal at P0 but over time the mutants exhibit smaller maxillary lengths (J:181120)
• the inframaxillary length and the posterior-anterior length are reduced (J:181120)
• shortened maxillary regions; anterior-posterior facial length is normal at P0 but over time the mutants exhibit smaller maxillary lengths (J:181120)
• the inframaxillary length and the posterior-anterior length are reduced (J:181120)
• dental occlusion is misaligned with severe midface hypoplasia requiring trimming of the lower incisors weekly (J:181120)
• dental occlusion is misaligned with severe midface hypoplasia requiring trimming of the lower incisors weekly (J:181120)
• the palatal dimensions are proportionally smaller, however the structural components are in tact (J:181120)
• mutants exhibit reduced proliferation in the palate shelves at E14.5 and in the adjacent palatal epithelium (J:181120)
• mutants exhibit reduced hyaluronic acid in the palate shelves at E14.5 (J:181120)
• mutants exhibit aberrant vascular pattering in the palate including reduced vascular branching at E14.5 and E15.5 (J:181120)
• the palatal dimensions are proportionally smaller, however the structural components are in tact (J:181120)
• mutants exhibit reduced proliferation in the palate shelves at E14.5 and in the adjacent palatal epithelium (J:181120)
• mutants exhibit reduced hyaluronic acid in the palate shelves at E14.5 (J:181120)
• mutants exhibit aberrant vascular pattering in the palate including reduced vascular branching at E14.5 and E15.5 (J:181120)
• at E14.5, palatal shelf elevation above the tongue occurs normally but palatal elongation is reduced anteriorly, whereas the posterior palate shelf length is unaffected (J:181120)
• mutants exhibit delayed palatal shelf elongation (J:181120)
• some E15.5 mutants show delayed palate shelf apposition and the persistence of the epithelial seam, however palate fusion occurs in all mutants (J:181120)
• at E14.5, palatal shelf elevation above the tongue occurs normally but palatal elongation is reduced anteriorly, whereas the posterior palate shelf length is unaffected (J:181120)
• mutants exhibit delayed palatal shelf elongation (J:181120)
• some E15.5 mutants show delayed palate shelf apposition and the persistence of the epithelial seam, however palate fusion occurs in all mutants (J:181120)
• palate shelf height is reduced in both the anterior and posterior regions (J:181120)
• palate shelf height is reduced in both the anterior and posterior regions (J:181120)
• midfacial hypoplasia resulting in a reduction in anterior facial width (J:181120)
• midfacial hypoplasia resulting in a reduction in anterior facial width (J:181120)

skeleton
• shortened maxillary regions; anterior-posterior facial length is normal at P0 but over time the mutants exhibit smaller maxillary lengths (J:181120)
• the inframaxillary length and the posterior-anterior length are reduced (J:181120)
• shortened maxillary regions; anterior-posterior facial length is normal at P0 but over time the mutants exhibit smaller maxillary lengths (J:181120)
• the inframaxillary length and the posterior-anterior length are reduced (J:181120)
• dental occlusion is misaligned with severe midface hypoplasia requiring trimming of the lower incisors weekly (J:181120)
• dental occlusion is misaligned with severe midface hypoplasia requiring trimming of the lower incisors weekly (J:181120)

behavior/neurological
• mutants exhibit poor feeding after 30 days of age due to malocclusion and require soft mouse chow (J:181120)
• mutants exhibit poor feeding after 30 days of age due to malocclusion and require soft mouse chow (J:181120)

cardiovascular system
• mutants exhibit aberrant vascular pattering in the palate; reduced vascular branching in the palate at E14.5 and E15.5, reduced vasculature organization and vessel size, poor vascular smooth muscle investment, and irregular vessel formation (J:181120)
• mutants exhibit aberrant vascular pattering in the palate; reduced vascular branching in the palate at E14.5 and E15.5, reduced vasculature organization and vessel size, poor vascular smooth muscle investment, and irregular vessel formation (J:181120)

digestive/alimentary system
• the palatal dimensions are proportionally smaller, however the structural components are in tact (J:181120)
• mutants exhibit reduced proliferation in the palate shelves at E14.5 and in the adjacent palatal epithelium (J:181120)
• mutants exhibit reduced hyaluronic acid in the palate shelves at E14.5 (J:181120)
• mutants exhibit aberrant vascular pattering in the palate including reduced vascular branching at E14.5 and E15.5 (J:181120)
• the palatal dimensions are proportionally smaller, however the structural components are in tact (J:181120)
• mutants exhibit reduced proliferation in the palate shelves at E14.5 and in the adjacent palatal epithelium (J:181120)
• mutants exhibit reduced hyaluronic acid in the palate shelves at E14.5 (J:181120)
• mutants exhibit aberrant vascular pattering in the palate including reduced vascular branching at E14.5 and E15.5 (J:181120)
• at E14.5, palatal shelf elevation above the tongue occurs normally but palatal elongation is reduced anteriorly, whereas the posterior palate shelf length is unaffected (J:181120)
• mutants exhibit delayed palatal shelf elongation (J:181120)
• some E15.5 mutants show delayed palate shelf apposition and the persistence of the epithelial seam, however palate fusion occurs in all mutants (J:181120)
• at E14.5, palatal shelf elevation above the tongue occurs normally but palatal elongation is reduced anteriorly, whereas the posterior palate shelf length is unaffected (J:181120)
• mutants exhibit delayed palatal shelf elongation (J:181120)
• some E15.5 mutants show delayed palate shelf apposition and the persistence of the epithelial seam, however palate fusion occurs in all mutants (J:181120)
• palate shelf height is reduced in both the anterior and posterior regions (J:181120)
• palate shelf height is reduced in both the anterior and posterior regions (J:181120)

Mouse Models of Human Disease
OMIM ID Ref(s)
Alagille Syndrome 1; ALGS1 118450 J:181120


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory