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Phenotypes Associated with This Genotype
Genotype
MGI:3040398
Allelic
Composition
Llgl1tm1Vv/Llgl1tm1Vv
Meox2tm1(cre)Sor/Meox2+
Genetic
Background
involves: 129 * 129S4/SvJaeSor * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Llgl1tm1Vv mutation (1 available); any Llgl1 mutation (63 available)
Meox2tm1(cre)Sor mutation (3 available); any Meox2 mutation (18 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• die within 24 hours after birth

cardiovascular system
• areas affected with rosette-like structures are rich in dilated blood vessels
• moderate hemorrhage in the ventricles of the brain, detected as early as E12.5

craniofacial
• dome-like shape of head

embryo
• the apical-junctional complex in the neuroepithelium of E10.5 mutants is disrupted; pseudostratified neuroepithelium in the wild-type embryos is replaced by stratified neuroepithelium in the mutant
• formation of neuroepithelial rosette-like structures similar to the neuroblastic rosettes in human primitive neuroectodermal tumors

nervous system
• areas affected with rosette-like structures are rich in dilated blood vessels
• moderate hemorrhage in the ventricles of the brain, detected as early as E12.5
• many neural progenitor cells fail to exit the cell cycle and differentiate, and instead, continue to proliferate and die by apoptosis
• neural progenitor cells exhibit a loss of cell polarity
• many neural progenitor cells fail to differentiate, and instead continue to proliferate
• the apical-junctional complex in the neuroepithelium of E10.5 mutants is disrupted; pseudostratified neuroepithelium in the wild-type embryos is replaced by stratified neuroepithelium in the mutant
• formation of neuroepithelial rosette-like structures similar to the neuroblastic rosettes in human primitive neuroectodermal tumors
• newborns develop severe hydrocephalus
• ventricles become dilated by E15.5
• lateral ventricles of newborns appear dilated due to accumulation of cerebrospinal fluid and increasing pressure
• expansion of the striatum region of the brain at E12.5, where the neuroepithelial cell layer extends into the zones normally occupied by differentiating cells and forms aberrant rosette-like structures
• ventricular surface of cerebral cortex is destroyed and the subventricular structures are severely damaged
• overall increase in the neural progenitor cell domain and decrease in the differentiated neuronal cell domain
• overall reduction of in the numbers of differentiated neurons in the striatum region

skeleton
• dome-like shape of head

cellular
• many neural progenitor cells fail to exit the cell cycle and differentiate, and instead, continue to proliferate and die by apoptosis
• neural progenitor cells exhibit a loss of cell polarity
• many neural progenitor cells fail to differentiate, and instead continue to proliferate


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
04/09/2024
MGI 6.23
The Jackson Laboratory