Symbol
Name
ID

Kcne1
potassium voltage-gated channel, Isk-related subfamily, member 1
MGI:96673

Phenotype annotations related to hearing/vestibular/ear

Darker colors indicate more annotations

Human Phenotypes	Congenital sensorineural hearing impairment
Disease(s) Associated with KCNE1	Congenital sensorineural hearing impairment
Jervell-Lange Nielsen syndrome

Mouse Phenotypes

absent vestibuloocular reflex

abnormal inner ear morphology

abnormal cochlea morphology

collapsed Reissner membrane

abnormal cochlear sensory epithelium morphology

absent cochlear hair cells

absent cochlear inner hair cells

absent cochlear outer hair cells

absent outer hair cell stereocilia

cochlear hair cell degeneration

abnormal scala media morphology

absent organ of Corti

organ of Corti degeneration

abnormal stria vascularis morphology

abnormal tectorial membrane morphology

small scala media

vestibular hair cell degeneration

abnormal semicircular canal morphology

abnormal crista ampullaris morphology

abnormal crista ampullaris neuroepithelium morphology

abnormal inner ear vestibule morphology

utricular macular degeneration

vestibular saccular macula degeneration

abnormal vestibular dark cell morphology

vestibular dark cell degeneration

abnormal otolith morphology

abnormal ear physiology

abnormal auditory brainstem response

increased or absent threshold for auditory brainstem response

deafness

abnormal vestibular system physiology

absent linear vestibular evoked potential

Availability

Mouse Genotype

Kcne1^2J/Kcne1^2J

Kcne1^pkr/Kcne1^pkr

Kcne1^{tm1b(EUCOMM)Hmgu}/Kcne1^{tm1b(EUCOMM)Hmgu}

Kcne1^tm1Sfh/Kcne1^tm1Sfh

Kcne1^pkr/Kcne1⁺

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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