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Phenotypes Associated with This Genotype
Genotype
MGI:4361520
Allelic
Composition
Tg(CAG-cat,-Ptpn11*Q97R)1Rbns/0
Tg(Wnt1-cre)11Rth/0
Genetic
Background
involves: C57BL/6J * CBA/J * FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CAG-cat,-Ptpn11*Q97R)1Rbns mutation (0 available)
Tg(Wnt1-cre)11Rth mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice that exhibit a cleft palate die within 5 days of birth

craniofacial
• mice exhibit craniofacial defects that become more pronounced with age
• however, treatment with U0126 restores skull size, length, mandibular bone length, inner canthal distance, and frontal bone height
• at E17.5 and P2, mice exhibit abnormal skull morphology
• however, treatment with U0126 restores skull size, length, mandibular bone length, inner canthal distance, and frontal bone height
• mice exhibit a reduced skull length compared to in wild-type mice
• however, treatment with U0126 restores frontal bone morphology
• mice exhibit larger than normal anterior fontanelle
• however, treatment with U0126 restores normal anterior fontanelle morphology
• mice exhibit taller frontal bone heights compared with wild-type mice
• however, treatment with U0126 restores frontal bone morphology
• proportional to the small skull length
• however, treatment with U0126 restores mandible length
• dome-shaped head
• in 21% of mice
• in 21% of mice
• nasal bones fails to fuse along the midline unlike in wild-type mice
• mice have a broad nose
• nasal cartilage fails to fuse along the midline unlike in wild-type mice

skeleton
• at E17.5 and P2, mice exhibit abnormal skull morphology
• however, treatment with U0126 restores skull size, length, mandibular bone length, inner canthal distance, and frontal bone height
• mice exhibit a reduced skull length compared to in wild-type mice
• however, treatment with U0126 restores frontal bone morphology
• mice exhibit larger than normal anterior fontanelle
• however, treatment with U0126 restores normal anterior fontanelle morphology
• mice exhibit taller frontal bone heights compared with wild-type mice
• however, treatment with U0126 restores frontal bone morphology
• proportional to the small skull length
• however, treatment with U0126 restores mandible length
• dome-shaped head
• nasal cartilage fails to fuse along the midline unlike in wild-type mice

growth/size/body
• in 21% of mice
• in 21% of mice
• nasal bones fails to fuse along the midline unlike in wild-type mice
• mice have a broad nose
• nasal cartilage fails to fuse along the midline unlike in wild-type mice
• mice exhibit a reduced body weight that is partially restored by treatment with U0126
• webbed neck
• mice exhibit a short stature that is partially restored by treatment with U0126
• pronounced at P2
• slightly at E17.5

embryo
• neural crest cells fail to contribute to the parietal bone unlike in wild-type mice

hearing/vestibular/ear

vision/eye
• mice exhibit hypetelorism and a greater inner canthal distance compared with wild-type mice
• however, treatment with U0126 restores inner canthal distance

digestive/alimentary system
• in 21% of mice

respiratory system
• nasal bones fails to fuse along the midline unlike in wild-type mice
• mice have a broad nose
• nasal cartilage fails to fuse along the midline unlike in wild-type mice

integument
• webbed neck

cellular
• neural crest cells fail to contribute to the parietal bone unlike in wild-type mice

Mouse Models of Human Disease
OMIM ID Ref(s)
Noonan Syndrome 1; NS1 163950 J:153094


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
09/27/2016
MGI 6.05
The Jackson Laboratory