Analysis Tools|
Allele Symbol Allele Name Allele ID |
Emx1tm1(cre)Yql targeted mutation 1, Yuqing Li MGI:2176219 |
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| Summary |
9 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• Background Sensitivity: the absence of a corpus collosum was noted on a segregating 129/Sv x C57BL/6 genetic background; however, no such phenotype was reported when the mutation was made congenic on a C57BL/6 background
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• females display poor nursing behavior
• 80% do not nurse at all and abandon pups
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• increased sensitivity to PTZ induced seizures
• 83% die within 60 minutes of PTZ injection compared to 58% mortality in controls
• significantly higher numbers of phase I and phase II seizures
• duration of seizures increased for phase I
• 2X as much time in seizure as controls
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• males significantly smaller than littermates
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• increased sensitivity to PTZ induced seizures
• 83% die within 60 minutes of PTZ injection compared to 58% mortality in controls
• significantly higher numbers of phase I and phase II seizures
• duration of seizures increased for phase I
• 2X as much time in seizure as controls
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• as a result of missing hippocampal structures
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• lack hippocampal commissure
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• CA1, CA2, CA3 all not detected
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• impaired cortical development, failure of lobes to extend caudally
• adult brain similar in appearance to perinatal brain
• thinner cortex
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit subtle to inapparent abnormalities in the cortical hem at E12.5
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• strong reduction in gephyrin cluster numbers in the hippocampus at P40, P60 and P130
• however, clustering is unaffected in other areas of the brain
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• impaired
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• in a Morris water maze
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• in a Morris water maze
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• increased depression-like behavior
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• mice exhibit increased latency to feed in a novelty suppressed feed test compared with wild-type mice
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• slight
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• at E18, Cux1+ neurons are mislocalized in the deep layers of the lateral somatosensory cortex compared to in wild-type mice
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• most cultured cortical neurons remain multipolar shape unlike control neurons
• axonal branching in the somatosensory cortex and the corticothalamic tracts is increased compared with control mice
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• on a beam-walking test, 71% of mice exhibit more falls than wild-type mice
• however, mice perform normally in a rotarod test
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• the hind base of male mice is smaller than in wild-type mice
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• mice exhibit hyperactivity in an open-field test
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| N |
• the cerebral cortex and dopamine metabolite levels are normal
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit a 219% increase of slips in the beam walking test
• however, mice do not show a deficit in the rotarod test
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• in the continuous open field test, mice show an increase in activity level during the light phase but not dark phase indicating a rest-phase specific motor restlessness
• however, mice exhibit a similar level of total distance traveled in a 30 min open field test as controls and similar wheel running activity during both the light and dark phase as in controls
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• sleep analysis indicates an increased probability of waking in the light phase but not dark phase
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• mice exhibit lower sensitivity to the heat stimuli than controls in the tail-flick test, indicating decreased thermal sensory perception
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• mice exhibit thinner cortical layers in the posterior primary motor cortex
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• mice exhibit thinner cortical layers in both the anterior and posterior part of the primary somatosensory cortex representing the hindlimb
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| restless legs syndrome | DOID:0050425 |
OMIM:PS102300 |
J:282582 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• behavior in Morris water maze, elevated plus maze, open field and context-dependent and cue-dependent fear conditioning tests
• locomotor behavior in Morris water maze, elevated plus maze and open field tests
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• significantly lower cumulative doses of PTZ and latency to induce onset of generalized tonic-clonic seizures with pentylenetetrazol (PTZ)-induced seizures
• significantly longer intervals between minimal and tonic-clonic seizures with pentylenetetrazol (PTZ)-induced seizures
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• many cells positive for Cux1 (marker for cerebral cortex layers II-IV) remained in the intermediate zone (IZ) at age P2
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• significantly lower cumulative doses of PTZ and latency to induce onset of generalized tonic-clonic seizures with pentylenetetrazol (PTZ)-induced seizures
• significantly longer intervals between minimal and tonic-clonic seizures with pentylenetetrazol (PTZ)-induced seizures
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• many cells positive for Cux1 (marker for cerebral cortex layers II-IV) remained in the intermediate zone (IZ) at age P2
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• at E16 and P0, mice exhibit normal overall structure of the cerebral cortex, with no significant differences in cortical thickness, corpus callosum and hippocampus morphology, or cortical layer organization relative to control mice
• no defects in cortical neuron migration are noted at P0; both neural stem cells and intermediate progenitor cells show norma proliferation patterns at E16; no change in the number of TUNEL+ apoptotic cells is noted in the cortex at E17.5
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• critical synaptic proteins, including NMDA receptor subunits GRIN1, GRIN2A and GRIN2B, are downregulated in the cerebral cortex of 2-month-old mice
• postsynaptic scaffold proteins DLG4 (also known as PSD95), SHANK3, and HOMER1 are downregulated in isolated synaptic fractions of the neocortex
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• E16 and P0 brain slices show abnormal Map2 and Tubb3 (also known as Tuj1) staining patterns with fragmented longitudinal stripes substantially different from control patterns
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• in vitro differentiation of dissociated E16 cortical neurons shows impaired neuronal process development, with a significant reduction in neurite length
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• Golgi-Coxstained cortical sections from P20- or 2-month-old mice show a significant reduction in apical dendrite length
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• Golgi-Coxstained apical dendrites in the cerebral cortex and hippocampus of P20- or 2-month-old mice show a significant reduction in dendritic spine density on apical dendrites
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• EM analysis of synapses in the prefrontal cortex of 2- to 4-month-old mice shows a significant reduction in both the length and thickness of the postsynaptic density
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• spontaneous glutamatergic excitatory postsynaptic currents (sEPSCs) of prefrontal cortex pyramidal neurons from P15- to P20-old mice show a significant increase in sEPSC peak amplitude
• however, no change in sEPSC amplitude is seen in prefrontal cortex slices from 4-month-old mice, suggesting that the effects on neuronal synaptic network activity may vary at different developmental stages
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• whole-cell voltage-clamp recordings of spontaneous glutamatergic excitatory postsynaptic currents (sEPSCs) show a significant decrease in sEPSC frequency of pyramidal neurons in prefrontal cortex slices from 4-month-old mice
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• sEPSCs of prefrontal cortex pyramidal neurons from P15- to P20-old mice show a significant increase in sEPSC frequency
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• average paired-pulse ratio (PPR) of evoked EPSCs is significantly increased at interpulse intervals of 25, 50, and 100 ms in prefrontal cortex pyramidal neurons from 2-month-old mice
• however, no change in average PPR is seen in pyramidal neurons from P15- to P20-old mice
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| N |
• mice show normal memory and cognition in the Morris water maze and novel object recognition test, normal instinctive behavior in a nest building test, and unaffected motor coordination/balance abilities in a balance beam test
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• in an open-field assay, mice spend less time in the central area and more time in the peripheral areas, with no significant change in total distance traveled or mean speed relative to controls
• however, in an elevated plus maze test, mice show only a small (non-significant) decrease in the percentage of time spent in the open arm
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• during stage 3 of a three-chamber test, mice show no preference for a novel stranger mouse (stranger 2), indicating impaired preference for social novelty
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• mice show increased self-grooming duration and frequency relative to controls
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• in a marble burying test, mice show increased marble burying behavior relative to control mice
• in a self-grooming test, mice show increased self-grooming duration and frequency during the 10 min free movement session
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• in a reciprocal social interactions test, 2- to 3-month-old mice spend significantly less time interacting with a newly introduced mouse than controls
• during stage 2 of a three-chamber test, mice spend more time in the empty compartment than exploring a stranger mouse (stranger 1), suggesting impaired social interaction
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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 03/25/2025 MGI 6.24 |
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