Annotation Detail

Information
Associated Genes
GJB2
Associated Variants
GJB2 p.Lys15Thr (p.K15T) ( ENST00000382844.2, ENST00000382848.5 )
GJB2 p.Lys15Thr (p.K15T) ( ENST00000382844.2, ENST00000382848.5 )
Associated Disease
Autosomal recessive nonsyndromic hearing loss 1A Mutilating keratoderma Ichthyosis, hystrix-like, with hearing loss X-linked mixed hearing loss with perilymphatic gusher palmoplantar keratoderma-deafness syndrome Autosomal dominant nonsyndromic hearing loss 3A Knuckle pads, deafness AND leukonychia syndrome Autosomal dominant keratitis-ichthyosis-hearing loss syndrome
Source Database
ClinVar
Description
NM_004004.6(GJB2):c.44A>C (p.Lys15Thr) AND multiple conditions
ClinVar Allele ID
53919
ClinVar RefSeq Alternation Syntax
NM_004004.6:c.44A>C
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2021-12-14
Clinical Significance Review Status
criteria provided, single submitter
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV002490513
ClinVar Disease
Palmoplantar keratoderma-deafness syndrome
ClinVar Disease
Knuckle pads, deafness AND leukonychia syndrome
ClinVar Disease
Autosomal dominant keratitis-ichthyosis-hearing loss syndrome
ClinVar Disease
Autosomal recessive nonsyndromic hearing loss 1A
ClinVar Disease
Mutilating keratoderma
ClinVar Disease
Ichthyosis, hystrix-like, with hearing loss
ClinVar Disease
X-linked mixed hearing loss with perilymphatic gusher
ClinVar Disease
Autosomal dominant nonsyndromic hearing loss 3A
Observed Origin Sample
unknown
Drugs