Annotation Detail

Information
Associated Genes
GJB2
Associated Variants
GJB2 p.Asp179Tyr (p.D179Y) ( ENST00000382844.2, ENST00000382848.5 )
GJB2 p.Asp179Asn (p.D179N) ( ENST00000382848.5, ENST00000382844.2 )
GJB2 p.Gly12Val (p.G12V) ( ENST00000382848.5, ENST00000382844.2 )
GJB2 p.Gly12Asp (p.G12D) ( ENST00000382848.5, ENST00000382844.2 )
GJB2 p.Asp179Tyr (p.D179Y) ( ENST00000382844.2, ENST00000382848.5 )
GJB2 p.Asp179Asn (p.D179N) ( ENST00000382844.2, ENST00000382848.5 )
GJB2 p.Gly12Val (p.G12V) ( ENST00000382844.2, ENST00000382848.5 )
GJB2 p.Gly12Asp (p.G12D) ( ENST00000382844.2, ENST00000382848.5 )
Associated Disease
Sensorineural Hearing Loss (disorder)
Source Database
DisGeNET
Description
The proband was a 3-month-old infant with a congenital profound sensorineural hearing loss; direct sequencing of the GJB2 revealed the presence of a c.35delG mutation in the heterozygous state and a heterozygous G>C transition at nucleotide 535 in trans; this novel mutation, called p.D179H, resulted in an aspartic acid to histidine change at codon 179.
Pubmed
23812555
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.344722343815082
Year of publication
2013
Drugs