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Phenotypes Associated with This Genotype
Genotype
MGI:6285755
Allelic
Composition
Glultm1.1Ncd/Glultm1.1Ncd
Emx1tm1(cre)Krj/Emx1+
Genetic
Background
B6.Cg-Glultm1.1Ncd Emx1tm1(cre)Krj
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Emx1tm1(cre)Krj mutation (2 available); any Emx1 mutation (34 available)
Glultm1.1Ncd mutation (0 available); any Glul mutation (33 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• increase in mortality after 3 weeks of age, with the highest mortality between 4 and 9 weeks and a survival ratio of about 70% at 9 weeks of age

behavior/neurological
• fewer fecal boli are produced by mice during open field test sessions, suggesting reduced anxiety
• 4-15 week old mice show alterations in locomotive activities in the open field test, with some mice being almost inactive while others show hypoactivity interrupted by bursts of sudden wild running
• mice begin to exhibit hypoactivity at when subjected to mild stimulation such as removing the cage lid or gentle knocking on the cate at postnatal week 3
• mice begin to exhibit periodic running fits when subjected to mild stimulation such as removing the cage lid or gentle knocking on the cate at postnatal week 3
• mice older than 6 weeks show facial automatisms with occasional falling and forelimb clonus, suggesting seizures
• intracranial EEG recordings of 4-28 week old mice indicate spontaneous recurrent seizures in 4 of 7 mice
• frequency, duration, and behavioral severity of seizures varies among mice, with a tendency of more frequent seizures with age, indicating the development of late-onset epileptic seizures

cardiovascular system
• blood vessels become dilated in the areas of the neocortex and of the hippocampus where neurodegeneration occurs
• MRI cerebrovascular reactivity (the relative increase in blood oxygen level dependent signal during a carbon dioxide challenge) is reduced in neocortical areas in both 4 week and 12-15 week old mice indicating that cerebral blood vessels exhibit reduced ability to react to increases in carbon dioxide levels
• MRI cerebrovascular reactivity is reduced in the dorsal hippocampus at 4 weeks but not 12-15 weeks of age

homeostasis/metabolism
• concentration of aspartate is lower in the cerebral cortex
• however, levels are normal in the cerebellum
• concentration of GABA is lower in the cerebral cortex
• however, levels are normal in the cerebellum
• concentration of glutamate is lower in the cerebral cortex
• however, levels are normal in the cerebellum
• concentration of glutamine are lower in the cerebral cortex
• however, levels are normal in the cerebellum

nervous system
• mice older than 6 weeks show facial automatisms with occasional falling and forelimb clonus, suggesting seizures
• intracranial EEG recordings of 4-28 week old mice indicate spontaneous recurrent seizures in 4 of 7 mice
• frequency, duration, and behavioral severity of seizures varies among mice, with a tendency of more frequent seizures with age, indicating the development of late-onset epileptic seizures
• blood vessels become dilated in the areas of the neocortex and of the hippocampus where neurodegeneration occurs
• with age, mice show selective degeneration of CA1 and CA3 pyramidal neurons
• mice exhibit progressive astrogliosis with a significant increase at 4 weeks of age, prior to neuronal loss and epilepsy
• astrogliosis is first noted in astrocytes along cerebral blood vessels
• some mice show occasional focal lesions (small islands of neuronal loss) in the neocortex at 6 weeks of age
• with age, mice show selective degeneration of CA1 and CA3 pyramidal neurons and dentate granule cells which is apparent by 3 months of age
• the neocortex becomes progressively affected in multiple areas with age, particularly the retrosplenial, motor, somatosensory, parietal association and visual cortices, with neocortical neurodegeneration generally starting in the most superficial layer of the cortex

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
temporal lobe epilepsy DOID:3328 OMIM:PS600512
J:272643


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