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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Cdh23v-bus
bustling waltzer
MGI:1856681
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Cdh23v-bus/Cdh23v-bus involves: KYF/MsIdr MGI:5140950


Genotype
MGI:5140950
hm1
Allelic
Composition
Cdh23v-bus/Cdh23v-bus
Genetic
Background
involves: KYF/MsIdr
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23v-bus mutation (1 available); any Cdh23 mutation (281 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• there is no response at any age
• hyperactivity interferes with care of offspring

hearing/vestibular/ear
• base-to-apex gradient severity of degeneration
• the first sign of degeneration is seen at 3 weeks of age in the outer hair cells of the basal turn of the cochlear
• TEM analyses of both the crista ampullaris and the macula showed hair deformities from E17 on
• abnormal patterns of kinocilium-stereocilia
• age-dependent degeneration
• SEM analysis revealed stereociliary derangements of both inner and outer hair cells by 10 days of age (J:34019)
• no normal stereocilia arrays are seen from 10 days to 6 months of age
• no normal stereocilia arrays are seen from 10 days to 6 months of age

nervous system
• SEM analysis revealed stereociliary derangements of both inner and outer hair cells by 10 days of age (J:34019)
• no normal stereocilia arrays are seen from 10 days to 6 months of age
• no normal stereocilia arrays are seen from 10 days to 6 months of age
• slow degeneration of spiral ganglion cells after 3 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Usher syndrome type 1D DOID:0110831 OMIM:601067
J:174130





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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
07/05/2024
MGI 6.24
The Jackson Laboratory