GenTIGSA Gene Database on Rare Genetic Disorders

Variants in ClinVar from 539 of 916 Rare Genetic Disorders of GenTIGS

Autosomal recessive nonsyndromic hearing loss 1A

An  Autosomal recessive, Digenic dominant  mode(s) within the Ear disorders  category

Likely pathogenic 1
Pathogenic 5
Pathogenic/Likely pathogenic 2

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_004004.6(GJB2):c.505T>C (p.Cys169Arg) Single nucleotide variant Chr13:20189077 Likely pathogenic Missense variant rs760489970 .Lifecell International Pvt. Ltd
NM_004004.6(GJB2):c.474C>G (p.Tyr158Ter) Single nucleotide variant Chr13:20189108 Pathogenic Nonsense rs375759781 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_004004.6(GJB2):c.94C>T (p.Arg32Cys) Single nucleotide variant Chr13:20189488 Pathogenic Missense variant rs371024165 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_004004.6(GJB2):c.313_326del (p.Lys105fs) Deletion Chr13:20189256 - 20189269 Pathogenic/Likely pathogenic rs111033253 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_004004.6(GJB2):c.551G>A (p.Arg184Gln) Single nucleotide variant Chr13:20189031 Pathogenic Missense variant rs80338950
NM_004004.6(GJB2):c.-23+1G>A Single nucleotide variant Chr13:20192782 Pathogenic/Likely pathogenic Splice donor variant rs80338940 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Lifecell International Pvt. Ltd
NM_004004.6(GJB2):c.231G>A (p.Trp77Ter) Single nucleotide variant Chr13:20189351 Pathogenic Nonsense rs80338944 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_001110219.3(GJB6):c.263C>T (p.Ala88Val) Single nucleotide variant Chr13:20223218 Pathogenic Missense variant rs28937872

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution