USH1G Chromosome 17

USH1 protein network component sans
67 variants 67 Health Risk

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What This Gene Does
This gene encodes a protein that contains three ankyrin domains, a class I PDZ-binding motif and a sterile alpha motif. The encoded protein interacts with harmonin, which is associated with Usher syndrome type 1C. This protein plays a role in the development and maintenance of the auditory and visual systems and functions in the cohesion of hair bundles formed by inner ear sensory cells. Mutations in this gene are associated with Usher syndrome type 1G (USH1G). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2013]
Gene Info
Gene Group
"Ankyrin repeat domain containing|Sterile alpha motif domain containing"
Locus Type
gene with protein product
Location
17q25.1
Ensembl
ENSG00000182040
Associated Conditions (13)
Inborn genetic diseases
USH1G-related disorder
Usher syndrome type 1G
Retinal dystrophy
Clear cell carcinoma of kidney
Optic atrophy
Hearing impairment
Usher syndrome
Usher syndrome type 1
Deafness
Hearing loss
autosomal recessive
Rare genetic deafness
Key Variants
All Variants (67)
RSID Category Clinical Significance Conditions
RS1187467461 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS138590256 Health Risk Conflicting classifications of pathogenicity USH1G-related disorder, USH1G-related disorder
RS1394276163 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS139897506 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, USH1G-related disorder, Retinal dystrophy
RS141688757 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS142486910 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, USH1G-related disorder, Retinal dystrophy
RS144633924 Health Risk Conflicting classifications of pathogenicity
RS145448362 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Optic atrophy, Usher syndrome type 1G
RS147967199 Health Risk Conflicting classifications of pathogenicity USH1G-related disorder, USH1G-related disorder
RS149529031 Health Risk Conflicting classifications of pathogenicity Hearing impairment, Usher syndrome type 1G, Hearing impairment
RS199648830 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS199724052 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS200082225 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS201123735 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Inborn genetic diseases, Usher syndrome type 1G
RS201359669 Health Risk Conflicting classifications of pathogenicity USH1G-related disorder, USH1G-related disorder
RS201644674 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, USH1G-related disorder, Usher syndrome type 1G
RS2038930076 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS374260443 Health Risk Conflicting classifications of pathogenicity
RS397517927 Health Risk Conflicting classifications of pathogenicity
RS544952984 Health Risk Conflicting classifications of pathogenicity Usher syndrome, Usher syndrome type 1G, Usher syndrome
RS55744500 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS569032124 Health Risk Conflicting classifications of pathogenicity Usher syndrome type 1G, Usher syndrome type 1G
RS776968656 Health Risk Conflicting classifications of pathogenicity Inborn genetic diseases, Inborn genetic diseases
RS1226851798 Health Risk Likely pathogenic Usher syndrome, Usher syndrome
RS1567940507 Health Risk Likely pathogenic Hearing impairment, Hearing impairment
RS2544431166 Health Risk Likely pathogenic USH1G-related disorder, USH1G-related disorder
RS727504890 Health Risk Likely pathogenic Usher syndrome, Usher syndrome
RS755908331 Health Risk Likely pathogenic
RS104894651 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS104894652 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS1316299165 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS1329606728 Health Risk Pathogenic
RS1359109336 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS1555627787 Health Risk Pathogenic Usher syndrome type 1, Usher syndrome type 1
RS1567941869 Health Risk Pathogenic
RS1598584825 Health Risk Pathogenic Usher syndrome type 1, Usher syndrome type 1
RS201866631 Health Risk Pathogenic Deafness, Usher syndrome type 1G, Hearing loss
RS2038909213 Health Risk Pathogenic
RS2144752958 Health Risk Pathogenic
RS2144753776 Health Risk Pathogenic
RS2144754031 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS2144754179 Health Risk Pathogenic
RS2144755152 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS2144755466 Health Risk Pathogenic
RS2144755488 Health Risk Pathogenic
RS2544427211 Health Risk Pathogenic
RS2544427345 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
RS2544427492 Health Risk Pathogenic
RS2544428980 Health Risk Pathogenic Usher syndrome, Usher syndrome
RS2544431739 Health Risk Pathogenic Usher syndrome type 1G, Usher syndrome type 1G
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