Analysis Tools|
Allele Symbol Allele Name Allele ID |
Atp1a3Myk Myshkin MGI:4356167 |
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| Summary |
7 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• maximal tonic hindlimb extension is followed by death
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• in the Porsolt forced swim test, mice spend a longer time active than wild-type mice, indicating increased motivation
(J:180250)
• chronic lithium carbonate and valproic acid treatment reduces the behavioral abnormalities of mutants
(J:180250)
• treatment with SL327, an inhibitor of ERK, and rostafuroxin, a compound that selectively displaces ouabain from the Na+,K+-ATPase reduces the behavioral abnormalities of mutants
(J:180250)
• mice take longer to find a hidden platform and swim further compared with wild-type mice
(J:262367)
• however, swim speed and floating is normal
(J:262367)
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• mice show increased sensitivity to low dose of D-amphetamine, showing increased ambulation, rearing, stereotypy, and circling behavior compared to wild-type mice
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• mice show a greater preference for reward compared to wild-type mice, consuming more sucrose solution relative to water, and initially consuming more sucrose solution before the choice test
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• mice do not habituate hole-board exploration
• the amount of rearing increases over time in mutants but decreases over time in wild-type mice, suggesting impaired habituation
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• mice have greater locomotor activity in the center compared with wild-type mice, suggesting decreased anxiety-like behavior
• in the elevated plus maze, mice make more open-arm entries and exploratory head dips and show a preference for the open arms
• in the light-dark box, mice spend a higher percentage of time in the light and do not show a preference for the dark compartment
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• in a Morris water maze due to reduced behavioral flexibility
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• mice explore objects and nosepoke more frequently than wild-type mice in the novel-object test and hole-board test
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• deficit in startle habituation
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• after training, mice on a balance beam exhibit more hind foot slips and take more time to cross than wild-type mice
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• walking path of mice is chaotic
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• in the novel open field, mice show hyperambulation, faster walking speed, and decreased freezing than wild-type mice
• mice are more hyperactive in the dark but not in response to light
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• mice entrain to light and shown normal circadian periods in a 12 hour light:12 hour dark environment, however, when external zeitgebers are removed, mice show an extended endogenous circadian period of 25 hours because of an increase in activity compared to 23.5 hours in wild-type mice
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• mice have more wake time than wild-type mice across 24 hours
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• REM sleep latency, as measured by the average duration of non-REM sleep that precedes entrance into REM sleep, is reduced
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• in the light phase, mice show shorter non-REM sleep bout length
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• in the light phase, mice show reduced number of REM sleep bouts
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• Background Sensitivity: while mutants backcrossed to C57BL/6NCr mice for 12 generations show increased susceptibility to stress-induced seizures, mutants backcrossed for 20 generations do not exhibit stress-induced seizure activity
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• 3 of 9 mice exhibit immediate induced convulsive seizure upon shaking of their cage
• however, treatment with valproic acid reduces cage-shaking-induced seizures and transfection with the wild-type copy of the gene prevents cage-shaking-induced seizures
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• at 4 weeks of age, mice exhibit spontaneous, recurrent behavioral convulsive seizures unlike wild-type mice
• seizures can be evoked by vestibular stress
• seizures begin with running and leaping, behavioral arrest, and Straub tail, are followed by whole body clonic jerking and falling, are accompanied by salivation, and occasionally progressed to maximal tonic hindlimb extension followed by death
• 3 of 9 mice exhibit immediate induced convulsive seizure upon shaking of their cage
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• during behavioral seizures mice exhibit spike-waves characteristic of epileptic discharge
• vestibular stress induces intermittent sharp waves that increase in amplitude until generalized bilateral synchronous tonic-clonic seizure activity is observed
• superfusion with magnesium ion free artificial cerebrospinal fluid induces repeated synchronized ictal bursts in the CA3 and entorhinal cortex of more than 50% of slices unlike in similarly treated wild-type slices
• after theta burst stimulation, mice exhibit enhanced excitation-spike coupling compared to wild-type mice
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| N |
• mice exhibit normal synaptic transmission and synaptic plasticity
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• Background Sensitivity: while mutants backcrossed to C57BL/6NCr mice for 12 generations show increased susceptibility to stress-induced seizures, mutants backcrossed for 20 generations do not exhibit stress-induced seizure activity
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• 3 of 9 mice exhibit immediate induced convulsive seizure upon shaking of their cage
• however, treatment with valproic acid reduces cage-shaking-induced seizures and transfection with the wild-type copy of the gene prevents cage-shaking-induced seizures
|
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• at 4 weeks of age, mice exhibit spontaneous, recurrent behavioral convulsive seizures unlike wild-type mice
• seizures can be evoked by vestibular stress
• seizures begin with running and leaping, behavioral arrest, and Straub tail, are followed by whole body clonic jerking and falling, are accompanied by salivation, and occasionally progressed to maximal tonic hindlimb extension followed by death
• 3 of 9 mice exhibit immediate induced convulsive seizure upon shaking of their cage
|
|
• during behavioral seizures mice exhibit spike-waves characteristic of epileptic discharge
• vestibular stress induces intermittent sharp waves that increase in amplitude until generalized bilateral synchronous tonic-clonic seizure activity is observed
• superfusion with magnesium ion free artificial cerebrospinal fluid induces repeated synchronized ictal bursts in the CA3 and entorhinal cortex of more than 50% of slices unlike in similarly treated wild-type slices
• after theta burst stimulation, mice exhibit enhanced excitation-spike coupling compared to wild-type mice
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• mice exhibit medial temporal sclerosis
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• mice exhibit aberrant membranous whorls in the hippocampal pyramidal neurons unlike in wild-type mice
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• neurons exhibit higher resting intracellular free calcium and show prolonged glutamate-evoked intracellular free calcium transients
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• deficit in prepulse inhibition
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• at 9 weeks
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• at 9 weeks
(J:151948)
• at 4 and 8 weeks in male and female mice
(J:262367)
• Background Sensitivity: unlike mice maintained on a mix background, female mice on a C57BL/6NCr congenic background exhibit decreased body weight
(J:262367)
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• increase in total brain Na+,K+-ATPase activity
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| alternating hemiplegia of childhood | DOID:0050635 |
OMIM:104290 OMIM:614820 OMIM:PS104290 |
J:262367 | |
| bipolar disorder | DOID:3312 | J:180250 , J:208021 | ||
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• maximal tonic hindlimb extension is followed by death
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| N |
• mice exhibit normal synaptic transmission and synaptic plasticity
|
|
• seizures can be evoked by vestibular stress
• mice exhibit spike wave discharge after cage shaking
• however, treatment with valproic acid reduces cage-shaking-induced seizures and transfection with the wild-type copy of the gene prevents cage-shaking-induced seizures
|
|
• at 4 weeks of age, mice exhibit spontaneous, recurrent behavioral convulsive seizures unlike wild-type mice
• seizures can be evoked by vestibular stress
• seizures begin with running and leaping, behavioral arrest, and Straub tail, are followed by whole body clonic jerking and falling, are accompanied by salivation, and occasionally progressed to maximal tonic hindlimb extension followed by death
• at 16 weeks, mice exhibit spontaneous seizures
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• mice exhibit numerous electrographic seizures unlike wild-type mice
• during behavioral seizures, mice exhibit spike-waves characteristic of epileptic discharge
• vestibular stress induces intermittent sharp waves that increase in amplitude until generalized bilateral synchronous tonic-clonic seizure activity is observed
• superfusion with magnesium ion free artificial cerebrospinal fluid induces repeated synchronized ictal bursts in the CA3 and entorhinal cortex of more than 50% of slices unlike in similarly treated wild-type slices
• after theta burst stimulation, mice exhibit enhanced excitation-spike coupling compared to wild-type mice
• mice exhibit spike wave discharge after cage shaking
|
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• mice exhibit medial temporal sclerosis
|
|
• mice exhibit aberrant membranous whorls in the hippocampal pyramidal neurons unlike in wild-type mice
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• at 9 weeks
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• at 9 weeks
|
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• seizures can be evoked by vestibular stress
• mice exhibit spike wave discharge after cage shaking
• however, treatment with valproic acid reduces cage-shaking-induced seizures and transfection with the wild-type copy of the gene prevents cage-shaking-induced seizures
|
|
• at 4 weeks of age, mice exhibit spontaneous, recurrent behavioral convulsive seizures unlike wild-type mice
• seizures can be evoked by vestibular stress
• seizures begin with running and leaping, behavioral arrest, and Straub tail, are followed by whole body clonic jerking and falling, are accompanied by salivation, and occasionally progressed to maximal tonic hindlimb extension followed by death
• at 16 weeks, mice exhibit spontaneous seizures
|
|
• during behavioral seizures, mice exhibit spike-waves characteristic of epileptic discharge
• vestibular stress induces intermittent sharp waves that increase in amplitude until generalized bilateral synchronous tonic-clonic seizure activity is observed
• superfusion with magnesium ion free artificial cerebrospinal fluid induces repeated synchronized ictal bursts in the CA3 and entorhinal cortex of more than 50% of slices unlike in similarly treated wild-type slices
• after theta burst stimulation, mice exhibit enhanced excitation-spike coupling compared to wild-type mice
• mice exhibit numerous electrographic seizures unlike wild-type mice
• mice exhibit spike wave discharge after cage shaking
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• Background Sensitivity: mice exhibit microglial activation unlike on a background lacking FVB/N
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• Background Sensitivity: mice exhibit astrogliosis unlike on a background lacking FVB/N
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• Background Sensitivity: mice exhibit microglial activation unlike on a background lacking FVB/N
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• Background Sensitivity: mice exhibit microglial activation unlike on a background lacking FVB/N
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• no mice were present at 4 weeks (time of death not given)
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• unlike Atp1a3Myk heterozygotes, mice exhibit normal coordination and contextual fear conditioning
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• mice take longer to find a hidden platform and swim further compared with wild-type mice
• however, swim speed and floating is normal
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• in a Morris water maze due to reduced behavioral flexibility
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| N |
• unlike Atp1a3Myk heterozygotes, mice exhibit normal body weight
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• cage shaking does not induce seizures as it does for Atp1a3Myk heterozygotes
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| N |
• mice perform at normal levels in the open field, elevated plus maze and light-dark box
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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 11/12/2024 MGI 6.24 |
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