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Phenotypes Associated with This Genotype
Genotype
MGI:3850961
Allelic
Composition
Gpsm2tm1.2Fuma/Gpsm2tm1.2Fuma
Genetic
Background
involves: C57BL/6 * C57BL/6NCrlj * CBA/JNCrlj
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gpsm2tm1.2Fuma mutation (0 available); any Gpsm2 mutation (26 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• mice do not exhibit a Preyers reflex
• mice do not exhibit a startle response to auditory stimulus

nervous system
N
• no apparent affect on neuronal production (J:132370)
• mice do not exhibit any overt differences in gross brain morphology compared to controls (J:230725)
• stereocilia of inner hair cells is short and stubby at P0, showing multiple rows of projections of uniform thickness instead of a single row of thick, long projections and shorter transient microvilli
• extra rows of stereocilia persist into adulthood and become short and stubby by P15, lacking the flexible tapered base and a staircase structure
• in outer hair cells, hair bundles are much flatter than the V-shaped stereocilia of controls
• 1.5-6.5% of outer hair cells show a spilt hair bundle in which 2 separate sterocilia clusters form at the apical surface of a single cell, with the kinocilium adjacent to only one cluster
• severity of hair bundle defects increases over time
• hair bundles are misoriented, with a broader distribution of hair bundle orientation in the 2nd and 3rd rows of outer hair cells
• an average of 11% of hair cells is misoriented
• orientation of apical divisions of the dorsolateral brain are randomized
• Pax6 expressing daughter cells appear in the subventricular zone

hearing/vestibular/ear
• stereocilia of inner hair cells is short and stubby at P0, showing multiple rows of projections of uniform thickness instead of a single row of thick, long projections and shorter transient microvilli
• extra rows of stereocilia persist into adulthood and become short and stubby by P15, lacking the flexible tapered base and a staircase structure
• in outer hair cells, hair bundles are much flatter than the V-shaped stereocilia of controls
• 1.5-6.5% of outer hair cells show a spilt hair bundle in which 2 separate sterocilia clusters form at the apical surface of a single cell, with the kinocilium adjacent to only one cluster
• severity of hair bundle defects increases over time
• hair bundles are misoriented, with a broader distribution of hair bundle orientation in the 2nd and 3rd rows of outer hair cells
• an average of 11% of hair cells is misoriented
• P17 mice show increased hearing threshold across all tested frequencies and an ABR response is not detected at the highest tested intensity of 100 dB
• mice are profoundly deaf at P17

cellular
• orientation of apical divisions of the dorsolateral brain are randomized
• results in Pax6 expressing daughter cells appearing in the subventricular zone


Contributing Projects:
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
03/19/2024
MGI 6.23
The Jackson Laboratory