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	Congenital sensorineural hearing impairment	Vestibular hypofunction	Absent vestibular function	Abnormal cochlea morphology
Disease(s) Associated with USH1C	Sensorineural hearing impairment	Congenital sensorineural hearing impairment	Vestibular hypofunction	Absent vestibular function	Abnormal cochlea morphology
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

Ush1c^dfcr-2J/Ush1c^dfcr-2J

Ush1c^dfcr-3J/Ush1c^dfcr-3J

Ush1c^dfcr-4J/Ush1c^dfcr-4J

Ush1c^dfcr/Ush1c^dfcr

Ush1c^tm1.1Mull/Ush1c^tm1.1Mull

Ush1c^tm1.1Ugds/Ush1c^tm1.1Ugds

Ush1c^{tm1a(KOMP)Wtsi}/Ush1c^{tm1a(KOMP)Wtsi}

Ush1c^tm1Xzl/Ush1c^tm1Xzl

Ush1c^tm1Xzl/Ush1c⁺

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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