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Phenotypes Associated with This Genotype
Genotype
MGI:3698722
Allelic
Composition
Espnje/Espnje
Genetic
Background
JE/LeJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Espnje mutation (2 available); any Espn mutation (34 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• the stereocilia degenerate shortly after birth
• the stereociliary degeneration appeared consistently more advanced in inner hair cells than that of outer hair cells
• although stereocilia bundles were present at birth, the length and width of stereocilia at P0 were reduced
• the stereocilia on outer hair cells were significantly shorter
• ABR assessment on homozygotes at 5 to 6 weeks of age shows no signs of hearing
• VESPs are absent at the maximum stimulus intensity used

behavior/neurological
• abnormal drop reflex; mice do not demonstrate expected dorsoflexion and spread out the front paws when quickly lowered from ~20 cm above a table surface, while controls do exhibit this behavior
• mice exhibit poor swimming ability; mice can not maneuver in the water and can not remain at the surface

nervous system
• the stereocilia degenerate shortly after birth
• the stereociliary degeneration appeared consistently more advanced in inner hair cells than that of outer hair cells
• although stereocilia bundles were present at birth, the length and width of stereocilia at P0 were reduced
• the stereocilia on outer hair cells were significantly shorter


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
03/25/2025
MGI 6.24
The Jackson Laboratory