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Symbol
Name
ID
Ush1c
USH1 protein network component harmonin
MGI:1919338
Phenotype annotations related to hearing/vestibular/ear
Darker colors indicate more annotations
Human Phenotypes
Sensorineural hearing impairment
Vestibular hypofunction
Absent vestibular function
Abnormal cochlea morphology
Disease(s) Associated with USH1C
autosomal recessive nonsyndromic deafness 18A
Usher syndrome type 1
Usher syndrome type 1C

Mouse Phenotypes
abnormal cochlear hair cell morphology
abnormal cochlear hair cell stereociliary bundle morphology
abnormal cochlear hair cell inter-stereocilial links morphology
abnormal cochlear hair bundle tip links morphology
abnormal inner hair cell stereociliary bundle morphology
abnormal orientation of inner hair cell stereociliary bundles
abnormal orientation of cochlear hair cell stereociliary bundles
abnormal orientation of outer hair cell stereociliary bundles
abnormal outer hair cell stereociliary bundle morphology
cochlear inner hair cell degeneration
abnormal cochlear outer hair cell morphology
cochlear outer hair cell degeneration
organ of Corti degeneration
vestibular hair cell degeneration
abnormal hair cell mechanoelectric transduction
increased or absent threshold for auditory brainstem response
deafness
Availability Mouse Genotype
Ush1cdfcr-2J/Ush1cdfcr-2J
Ush1cdfcr-3J/Ush1cdfcr-3J
Ush1cdfcr/Ush1cdfcr
Ush1ctm1.1Mull/Ush1ctm1.1Mull
Ush1ctm1.1Ugds/Ush1ctm1.1Ugds
Ush1ctm1a(KOMP)Wtsi/Ush1ctm1a(KOMP)Wtsi
Ush1ctm1Xzl/Ush1ctm1Xzl
Ush1ctm1Xzl/Ush1c+

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/12/2019
MGI 6.13
The Jackson Laboratory