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Phenotypes Associated with This Genotype
Genotype
MGI:3620098
Allelic
Composition
Kcnq4tm1.2Tjj/Kcnq4tm1.2Tjj
Genetic
Background
involves: 129/Sv * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Kcnq4tm1.2Tjj mutation (0 available); any Kcnq4 mutation (36 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• later than 4 weeks of age, selective degeneration of outer hair cells of the basal turn is observed; this progresses over time
• 1 year old animals exhibit a near complete loss of outer hair cells
• the IK,n current in the OHCs is abolished; the ensuing depolarization of OHCs impairs sound amplification
• at 42 weeks animals display a 50-60 dB shift in threshold compared to age-matched wild-type
• at P14 animals have a higher hearing threshold than controls but at P21 the hearing ability at those frequencies approaches that of controls
• DPOAEs could be recorded from 3-week-old mutant mice, albeit with lower amplitude
• At 8-10 weeks of age, DPOAEs were not distinguishable from the noise floor in mutant mice, but were readily observed in controls
• after P21, animals develop significant hearing loss

nervous system
• later than 4 weeks of age, selective degeneration of outer hair cells of the basal turn is observed; this progresses over time
• 1 year old animals exhibit a near complete loss of outer hair cells
• the IK,n current in the OHCs is abolished; the ensuing depolarization of OHCs impairs sound amplification


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
04/09/2024
MGI 6.23
The Jackson Laboratory