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Phenotypes Associated with This Genotype
Genotype
MGI:6110828
Allelic
Composition
H2az2Tg(Wnt1-cre)11Rth/H2az2+
Tg(CAG-Bmp4,-EGFP)1Ypc/0
Genetic
Background
involves: C57BL/6J * CBA/J * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2az2Tg(Wnt1-cre)11Rth mutation (2 available); any H2az2 mutation (26 available)
Tg(CAG-Bmp4,-EGFP)1Ypc mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
craniofacial
• slight decrease of cell proliferation rate in the maxillo-mandibular junction at E12.5
• ectopic cell apoptosis in the maxillo-mandibular junction at E12.5
• temporomandibular joint agenesis
• bony fusion of the jaws resembling congenital bony syngnathia is seen as early as E16.5
• the bony structures connecting the maxilla and mandible appear to derive from the posterior portion of the maxilla
• patterning of the secondary palate is disrupted; the anterior portion of the palatal shelves do not grow out from the maxilla at E13.5, and instead, a pair of palatal shelf-like structures originating from the mandible are seen, in a reverse direction with their tip pointing dorsally toward the nasal septum
• at E16.5, the palatal shelf-like structures remain separated and pointing dorsally along the tongue at the anterior level
• the mandibular protrusions are of palatal tissue origin
• palate shelves appear smaller at the posterior levels
• palate shelves are abnormally fused with the mandible

cellular
• increase in alkaline phosphatase (ALP) activity in the mandible and maxillo-mandibular junction at E12.5 and E13.5 indicating enhanced osteoblast differentiation

digestive/alimentary system
• patterning of the secondary palate is disrupted; the anterior portion of the palatal shelves do not grow out from the maxilla at E13.5, and instead, a pair of palatal shelf-like structures originating from the mandible are seen, in a reverse direction with their tip pointing dorsally toward the nasal septum
• at E16.5, the palatal shelf-like structures remain separated and pointing dorsally along the tongue at the anterior level
• the mandibular protrusions are of palatal tissue origin
• palate shelves appear smaller at the posterior levels
• palate shelves are abnormally fused with the mandible

growth/size/body
• patterning of the secondary palate is disrupted; the anterior portion of the palatal shelves do not grow out from the maxilla at E13.5, and instead, a pair of palatal shelf-like structures originating from the mandible are seen, in a reverse direction with their tip pointing dorsally toward the nasal septum
• at E16.5, the palatal shelf-like structures remain separated and pointing dorsally along the tongue at the anterior level
• the mandibular protrusions are of palatal tissue origin
• palate shelves appear smaller at the posterior levels
• palate shelves are abnormally fused with the mandible

hearing/vestibular/ear

skeleton
• increase in alkaline phosphatase (ALP) activity in the mandible and maxillo-mandibular junction at E12.5 and E13.5 indicating enhanced osteoblast differentiation
• slight decrease of cell proliferation rate in the maxillo-mandibular junction at E12.5
• ectopic cell apoptosis in the maxillo-mandibular junction at E12.5
• temporomandibular joint agenesis
• bony fusion of the jaws resembling congenital bony syngnathia is seen as early as E16.5
• the bony structures connecting the maxilla and mandible appear to derive from the posterior portion of the maxilla

vision/eye

respiratory system

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
cleft palate-lateral synechia syndrome DOID:0080313 OMIM:119550
J:214763


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
04/23/2024
MGI 6.23
The Jackson Laboratory