Phenotypes associated with this allele
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Eif2b4tm1.1Vdk mutation
(0 available);
any
Eif2b4 mutation
(40 available)
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mortality/aging
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• mice survive until 18-20 months of age
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growth/size/body
behavior/neurological
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• mice develop progressive gait ataxia
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• mice exhibit sporadic epileptic seizures
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nervous system
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• mice exhibit sporadic epileptic seizures
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• mice show onset of clinical vanishing white matter features around 7 months of age
• however, white matter does not contain myelin vacuoles
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• mice have abnormal white matter astrocytes from 2 months of age
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vision/eye
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• mice show signs of retinal laminar disorganization
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Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Eif2b4tm1.1Vdk mutation
(0 available);
any
Eif2b4 mutation
(40 available)
Eif2b5tm1.1Vdk mutation
(0 available);
any
Eif2b5 mutation
(31 available)
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mortality/aging
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• mice survive less than 3 weeks
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growth/size/body
behavior/neurological
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• mice develop progressive gait ataxia
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• mice exhibit sporadic epileptic seizures
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nervous system
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• mice exhibit sporadic epileptic seizures
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• white matter contains increased oligodendrocyte progenitor cell numbers
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• mice show vanishing white matter disease signs from P10
• vacuolization of the cerebellar white matter in P21 mice
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• hyaluronan is increased in P21 old forebrain
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• almost all Bergmann glia in P21 mice are mislocalized to the molecular layer of the cerebellar cortex and are GFAPdelta-immunopositive
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• white matter shows vacuolization; vacuoles are surrounded by myelin strands indicating that they are intramyelinic
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• the number of mature oligodendrocytes is deceased
• however, oligodendrocyte morphology is normal
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• deficient myelin formation, maturation, and maintenance, and progressive myelin vacuolization
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vision/eye
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• mice show signs of retinal laminar disorganization at 3 weeks of age
• retinal changes include uneven margins of the inner and outer nuclear layers with a thinned inner plexiform layer, ectopic inner nuclear cells, and displaced granule cells from the outer nuclear layer to the photoreceptor layer
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• uneven margins of the inner nuclear layer
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• uneven margins of the outer nuclear layer
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cellular
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• white matter contains increased oligodendrocyte progenitor cell numbers
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homeostasis/metabolism
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Eif2b4tm1.1Vdk mutation
(0 available);
any
Eif2b4 mutation
(40 available)
Eif2b5tm1.1Vdk mutation
(0 available);
any
Eif2b5 mutation
(31 available)
|
|
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mortality/aging
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• average lifespan is 4-5 months of age
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growth/size/body
behavior/neurological
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• mice develop progressive gait ataxia
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• mice exhibit sporadic epileptic seizures
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nervous system
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• mice exhibit sporadic epileptic seizures
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• mice exhibit vanishing white matter disease onset at 6 weeks of age
• vacuolization of the cerebellar white matter in 4 month old mice
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• white matter shows vacuolization; vacuoles are surrounded by myelin strands indicating that they are intramyelinic
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• mice have abnormal white matter astrocytes from 4 months of age
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• the corpus callosum shows decreased mean axonal diameter, with an increased percentage of small-caliber axons at 4 months of age
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• 4 month old mice show a paucity of myelin with pronounced vacuolization in the white matter
• deficient myelin formation, maturation, and maintenance
|
vision/eye
|
• mice show signs of retinal laminar disorganization
|
|
Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Eif2b4tm1.1Vdk mutation
(0 available);
any
Eif2b4 mutation
(40 available)
Eif2b5tm1.1Vdk mutation
(0 available);
any
Eif2b5 mutation
(31 available)
|
|
|
mortality/aging
|
• average lifespan is 4-5 months of age
|
growth/size/body
behavior/neurological
|
• mice develop progressive gait ataxia
|
|
• mice exhibit sporadic epileptic seizures
|
nervous system
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• mice exhibit sporadic epileptic seizures
|
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• mice exhibit vanishing white matter disease onset at 6 weeks of age
• vacuolization of the cerebellar white matter in 4 month old mice
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• white matter shows vacuolization; vacuoles are surrounded by myelin strands indicating that they are intramyelinic
|
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• mice have abnormal white matter astrocytes from 4 months of age
|
|
• the corpus callosum shows decreased mean axonal diameter, with an increased percentage of small-caliber axons at 4 months of age
|
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• 4 month old mice show a paucity of myelin with pronounced vacuolization in the white matter
• deficient myelin formation, maturation, and maintenance
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vision/eye
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• mice show signs of retinal laminar disorganization
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