Phenotypes associated with this allele
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grid2Lc-J mutation
(1 available);
any
Grid2 mutation
(85 available)
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mortality/aging
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• no mice are alive by 25 days of age
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• a portion of newborns die by 12 hours after birth
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homeostasis/metabolism
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grid2Lc-J mutation
(1 available);
any
Grid2 mutation
(85 available)
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Pyknotic Purkinjie cells in Grid2Lc-J/Grid2+ mouse cerebellum
behavior/neurological
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• first observed at 13 days of age
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• unable to maintain a rearing posture even when supported
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• mice may show head but not body movement
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• apparent by 2 weeks of age
• mice intermittently sway and lurch forward
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nervous system
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• hematoxylin and eosin staining of histological sections show significant degeneration of Purkinje cells in this area
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• precedes appearance of ataxic gait
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Allelic
Composition |
Grid2Lc-J/Grid2+
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Genetic
Background |
involves: 129X1/SvJ * BALB/cByJ * C57BL/6 |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grid2Lc-J mutation
(1 available);
any
Grid2 mutation
(85 available)
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behavior/neurological
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• a swaying of the body and tendancy to fall from side to side is found in the third week of life and persists in the adult
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nervous system
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• sparsely populated, atrophic lobes
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• at 30 days of age there are only 6% of normal numbers of Purkinje cells and these are not aligned in a monolayer
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• the Purkinje cells have stunted, poorly developed dendritic trees that fail to reach the pial surface
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• the cerebellum has only 10% of normal numbers of granule cells at 30 days of age
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• cerebellar atrophy is found by 30 days of age and persists
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behavior/neurological
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• disruption of Trp53 does not prevent or change the onset of the lurcher ataxia, which is found in the thrid week of life
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behavior/neurological
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• a swaying ataxia is found in the third week of age, the same as in simple lurcher heterozygotes
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nervous system
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• disruption of Bax rescues the granule cell death normally found in Lurcher mice such that these mice have a nearly normal, large cell-dense inner granule cell layer and normal lobular pattern of the cerebellar cortex
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• at 30 days of age immunohistochemistry shows that the Purkinje cells are not aligned in a monolayer and have stunted, poorly developed dendritic trees that fail to reach the pial surface, similar to the morphology found in Bax wild-type lurcher heterozygotes
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• there are few Purkinje cells at the interface of the inner granule cell layer and molecular layer, and, although cell counts show that there are 15% of normal numbers at 30 days of age, which is significantly more than the 6% normal level found in Bax wild-type lurcher heterozygotes, by 300 days of age the double mutant mice have only 1% of normal Purkinje cell numbers
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• although there are fewer than normal granule cells per midsagittal cerebellar section, there are approximately 60% of normal numbers which is a significant increase over the Bax wild-type lurcher heterozygotes
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Allelic
Composition |
Baxtm1Sjk/Bax+
Grid2Lc-J/Grid2+
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Genetic
Background |
involves: 129X1/SvJ * BALB/cByJ * C57BL/6 |
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behavior/neurological
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• a swaying ataxia is found in the third week of age, the same as in simple lurcher heterozygotes
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nervous system
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• gaps in the Purkinje cell layer found by 15 days of age
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• much smaller internal granule cell layer as early as 15 days of age
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Analysis Tools