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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Prkab1Gt(RRR454)Byg
gene trap RRR454, BayGenomics
MGI:3838355
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Prkab1Gt(RRR454)Byg/Prkab1Gt(RRR454)Byg involves: 129P2/OlaHsd * C57BL/6 MGI:3838360


Genotype
MGI:3838360
hm1
Allelic
Composition
Prkab1Gt(RRR454)Byg/Prkab1Gt(RRR454)Byg
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prkab1Gt(RRR454)Byg mutation (1 available); any Prkab1 mutation (18 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

nervous system
• mice exhibit seizures that recur within less than 10 minutes
• at E14.5, fewer cells in the forebrain undergoing mitosis and more Tuj1+ and Oligo2+ cells undergoing apoptosis compared to in wild-type mice
• large numbers of cells in the forebrain at E14.5 and cerebellum at P7 undergo apoptosis unlike in wild-type mice
• however, no cell death is observed in astrocytes at P7 and E18.5
• in culture, neurospheres exhibit increased cell death comapre4d to wild-type cells
• in culture, neurospheres produce fewer Tuj1+ neurons and O4+oligodendrocytes compared to wild-type neurospheres
• in culture, neurosphere differentiation into GFAP+ astrocytes is abnormal
• cultured cerebellar granule cell precursors from P2 cerebellum exhibit impaired reaggregate formation and neurite projection compared to wild-type cells
• however, cultured neurospheres transfected with a constitutively active Prkab1 exhibit rescued self-renewal and cell survival
• at E14.5, fewer cells in the forebrain undergoing mitosis and more Tuj1+ and Oligo2+ cells undergoing apoptosis compared to in wild-type mice
• at E18.5, 22% fewer mitotic cells around the lateral ventricles compared to in wild-type mice
• at P7, fewer cells in the dentate gyrus and cerebellum undergoing mitosis compared to in wild-type mice
• in culture, neurospheres are smaller than those that develop from wild-type cells
• proliferation of neuronal precursor cells is half of wild-type
• neuronal precursor cells exhibit defective mitosis in the G2M phase compared to wild-type cells
• at E18.5 and P14, brain size is reduced by 50% compared to in wild-type
• the dentate gyrus is nearly completely lost
• mice exhibit between 35% and 65% loss in cortical and granule neurons in the dentate gyrus compared to in wild-type mice
• reduction in the cerebral cortex results in improper cortical fusion and exposure of the superior and inferior colliculi unlike in wild-type mice
• mice exhibit disordered laminar organization in the cerebellum unlike in wild-type mice
• severe cerebellar atrophy
• mice exhibit spongiform vacuolation
• the number of astrocytes is increased compared to in wild-type mice
• at E18.5, the number of GFAP+ migrating astrocytes is greater than in wild-type mice
• extensive
• at P14, mice exhibit a 75% to 80% loss in oligodendrocytes compared to in wild-type mice
• mice exhibit loss of white matter axonal projections unlike in wild-type mice
• mice exhibit widespread loss of dendritic processes unlike in wild-type mice
• mice exhibit loss of neurons in the cortex, cerebellum, dentate gyrus, and hypothalamus compared to in wild-type mice
• mice exhibit between 35% and 65% loss in cortical and granule neurons in the inner granule layer compared to in wild-type mice
• translucent and hypomyelinated
• 30% thinner than in wild-type mice
• mice exhibit severe hypomyelination throughout the brain, particularly in the corpus callosum and striatum, compared to in wild-type mice
• the optic nerve is hypomyelinated

behavior/neurological
• severe
• mice exhibit an ataxic gait
• mice exhibit seizures that recur within less than 10 minutes

growth/size/body
• mice are emaciated at P14

vision/eye
• translucent and hypomyelinated
• 30% thinner than in wild-type mice

cellular
• at E14.5, fewer cells in the forebrain undergoing mitosis and more Tuj1+ and Oligo2+ cells undergoing apoptosis compared to in wild-type mice
• large numbers of cells in the forebrain at E14.5 and cerebellum at P7 undergo apoptosis unlike in wild-type mice
• however, no cell death is observed in astrocytes at P7 and E18.5
• in culture, neurospheres exhibit increased cell death comapre4d to wild-type cells
• in culture, neurospheres produce fewer Tuj1+ neurons and O4+oligodendrocytes compared to wild-type neurospheres
• in culture, neurosphere differentiation into GFAP+ astrocytes is abnormal
• cultured cerebellar granule cell precursors from P2 cerebellum exhibit impaired reaggregate formation and neurite projection compared to wild-type cells
• however, cultured neurospheres transfected with a constitutively active Prkab1 exhibit rescued self-renewal and cell survival
• at E14.5, fewer cells in the forebrain undergoing mitosis and more Tuj1+ and Oligo2+ cells undergoing apoptosis compared to in wild-type mice
• at E18.5, 22% fewer mitotic cells around the lateral ventricles compared to in wild-type mice
• at P7, fewer cells in the dentate gyrus and cerebellum undergoing mitosis compared to in wild-type mice
• in culture, neurospheres are smaller than those that develop from wild-type cells
• proliferation of neuronal precursor cells is half of wild-type
• neuronal precursor cells exhibit defective mitosis in the G2M phase compared to wild-type cells





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last database update
05/10/2022
MGI 6.19
The Jackson Laboratory