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Phenotypes Associated with This Genotype
Genotype
MGI:5697079
Allelic
Composition
Ercc8tm1Jhjh/Ercc8tm1Jhjh
Genetic
Background
B6J.129P2-Ercc8tm1Jhjh
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ercc8tm1Jhjh mutation (0 available); any Ercc8 mutation (31 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• gradient of inner hair cell loss from base to apex at 16 weeks of age
• complete loss of outer hair cells in the basal and middle turns of the cochlea at 16 weeks of age
• outer hair cells are more severely affected than inner hair cells
• mice show an increase in hearing thresholds at more than 8kHz at 13 weeks of age and thresholds remain elevated at 16 weeks
• auditory shifts are more more pronounced at 24 and 32 kHz than at 16 and 8 kHz
• DPOAEs are reduced in amplitude at 12 and 16 kHz or absent at 24 kHz by 16 weeks of age
• mice show progressive hearing loss

nervous system
• gradient of inner hair cell loss from base to apex at 16 weeks of age
• complete loss of outer hair cells in the basal and middle turns of the cochlea at 16 weeks of age
• outer hair cells are more severely affected than inner hair cells

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Cockayne syndrome DOID:2962 J:219993


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
04/30/2024
MGI 6.23
The Jackson Laboratory