Annotation Detail

Information
Associated Genes
GJB2
Associated Variants
GJB2 p.His73Arg (p.H73R) ( ENST00000382848.5, ENST00000382844.2 )
GJB2 p.His73Arg (p.H73R) ( ENST00000382844.2, ENST00000382848.5 )
Associated Disease
Mutilating keratoderma
Source Database
DisGeNET
Description
Here, we report on a family with a novel GJB2 mutation (p.His73Arg) causing a syndrome of focal palmoplantar keratoderma with severe progressive sensorineural hearing impairment, a phenotype reminiscent of Vohwinkel syndrome.
Pubmed
17993581
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.482171534976642
Year of publication
2008
Drugs