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Phenotypes Associated with This Genotype
Genotype
MGI:5140886
Allelic
Composition
Cdh23Jera/Cdh23Jera
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23Jera mutation (0 available); any Cdh23 mutation (81 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
N
• no signs of vestibular dysfunction
• the tunnel of Corti is less well formed at 4 weeks of age
• absence of stereociliary tip tenting of IHC second tallest row stereocilia indicating disruption of tip links
• some indication of hair cell degeneration in the basal cochlear region at 4 weeks of age
• by 8 and 12 weeks of age signs of hair cell degeneration are clearly apparent in the basal and midcochlear turns
• collapse of the organ of Corti in the basal and midcochlear turns at 8 and 12 weeks of age
• significantly increased threshold at 4 weeks of age
• profound hearing loss at 4 weeks of age

behavior/neurological
N
• no signs of vestibular dysfunction

nervous system
• absence of stereociliary tip tenting of IHC second tallest row stereocilia indicating disruption of tip links
• some indication of hair cell degeneration in the basal cochlear region at 4 weeks of age
• by 8 and 12 weeks of age signs of hair cell degeneration are clearly apparent in the basal and midcochlear turns
• loss of spiral ganglion neurons in the basal and midcochlear turns at 8 and 12 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
autosomal recessive nonsyndromic deafness 12 DOID:0110467 OMIM:601386
J:174130


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
10/08/2019
MGI 6.14
The Jackson Laboratory