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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Grid2ho-4J
hotfoot 4 Jackson
MGI:1856050
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Grid2ho-4J/Grid2ho-4J DBA/2J-Grid2ho-4J/J MGI:3698846
ht2
Grid2ho-4J/Grid2ho-Tg37Inra involves: C57BL/6 * CBA * DBA/2J MGI:3834101
cx3
Bcl2l2Gt(ROSA)41Sor/Bcl2l2Gt(ROSA)41Sor
Grid2ho-4J/Grid2ho-4J
involves: 129S5/SvEvBrd * DBA/2J MGI:3834108


Genotype
MGI:3698846
hm1
Allelic
Composition
Grid2ho-4J/Grid2ho-4J
Genetic
Background
DBA/2J-Grid2ho-4J/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grid2ho-4J mutation (1 available); any Grid2 mutation (85 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• elevated threshold and reduced amplitudes

behavior/neurological
• mice fail to improve performance in rotating rod trials in contrast to wild-type animals (J:137190)
• in a non-rotating rod test, equilibrium times of mutants are lower than controls in the first trial, but not the 2nd or 3rd trial
• mutants exhibit poor performance in a motor training test with a rotarod, and are unable to improve their performance
• tapping of the hindpaws; phenotype is much less severe than in Grid2ho-Nancy mice
• mutants exhibit increased stumbling frequency in a motor coordination test

cellular
• mitochondria lengths in Purkinje cells are 1.3-1.6 um, compared to lengths of 0.8-1.0 um in wild-type animals
• often, points where mitochondria become constricted are observed in mutants but not in wild-type mitochondria, suggestive of impaired fission

nervous system
• numerous clusters of naked spines are located in the molecular layer around secondary branches and spiny branchlets of Purkinje cell dendrites
• mitochondria lengths in Purkinje cells are 1.3-1.6 um, compared to lengths of 0.8-1.0 um in wild-type animals
• often, points where mitochondria become constricted are observed in mutants but not in wild-type mitochondria, suggestive of impaired fission
• Purkinje cell dendrites have ectopic spines, some of which are naked and bore very long postsynaptic differentiations directly apposed to astrocytic processes
• occasional appositions between a Purkinje cell dendritic spine and two parallel fiber varicosities
• some granule cell pyknotic nuclei in the granule cell layer
• although most Purkinje cells are innervated by a single climbing fiber, about 19-20% of cells show innervation by more than one climbing fiber
• mismatching between the length of the presynaptic active zones, in parallel fiber varicosities, and the postsynaptic differentiations in Purkinje cell dendritic spines




Genotype
MGI:3834101
ht2
Allelic
Composition
Grid2ho-4J/Grid2ho-Tg37Inra
Genetic
Background
involves: C57BL/6 * CBA * DBA/2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Grid2ho-4J mutation (1 available); any Grid2 mutation (85 available)
Grid2ho-Tg37Inra mutation (0 available); any Grid2 mutation (85 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice exhibit jerky movements of the hindlimbs, similar to what is observed in the Grid2ho homozygotes




Genotype
MGI:3834108
cx3
Allelic
Composition
Bcl2l2Gt(ROSA)41Sor/Bcl2l2Gt(ROSA)41Sor
Grid2ho-4J/Grid2ho-4J
Genetic
Background
involves: 129S5/SvEvBrd * DBA/2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bcl2l2Gt(ROSA)41Sor mutation (2 available); any Bcl2l2 mutation (19 available)
Grid2ho-4J mutation (1 available); any Grid2 mutation (85 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• smaller than wild-type or single-mutant animals

behavior/neurological
• when manually induced to move, animals respond with a very ataxic gait
• due to severity of ataxia, mice could not be tested on rotating rod apparatus
• mice exhibit very little movement relative to wild-type or single mutants

nervous system
• cerebellum is smaller than wild-type or single mutant mice
• layer is overcrowded in the smaller cerebellum compared to controls, however Purkinje cell numbers are not different
• approximately 45% of cells analyzed have 2 dendritic branches instead of the single primary branch seen in wild-type
• mitochondria lengths in Purkinje cell dendrites are 1.8-1.9 um, significantly longer than those of either single mutant or wild-type
• frequently, mitochondria contain sites of thinning and constriction; in some instances, cristae in mitochondria appear slightly dilated
• extremely long mitochondria that extend for much of the visible length of the dendrites are frequently seen
• large numbers of naked dendritic spines are present, similar in number to Bcl2l2Gt(ROSA)41Sor mice
• dendritic arbors are reduced in number and complexity relative to controls
• numbers of branches in arbors of each level of complexity are decreased
• spacing of dendritic spines is abnormal, with spines crowded onto dendrites in contrast to regular spacing in wild-type and single mutant cells
• spines are shorter and often are branched or lack a characteristic spine head or neck
• mismatched connections between pre- and postsynaptic active zones of Purkinje cell/parallel fiber synapses are frequently seen
• other synaptic defects such as shortened active zones and thickened postsynaptic densities

cellular
• mitochondria lengths in Purkinje cell dendrites are 1.8-1.9 um, significantly longer than those of either single mutant or wild-type
• frequently, mitochondria contain sites of thinning and constriction; in some instances, cristae in mitochondria appear slightly dilated
• extremely long mitochondria that extend for much of the visible length of the dendrites are frequently seen





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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory