Annotation Detail

Information
Associated Genes
KCNQ1
Associated Variants
KCNQ1 p.Arg518Gly (p.R518G) ( ENST00000335475.6, ENST00000155840.12, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Arg518Ter (p.R518*) ( ENST00000335475.6, ENST00000155840.12, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Arg518Gly (p.R518G) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Arg518Ter (p.R518*) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
Associated Disease
Jervell-Lange Nielsen syndrome
Source Database
DisGeNET
Description
The R518X/KCNQ1 mutation is a common cause of autosomal recessive (Jervell and Lange Nielsen Syndrome- JLNS) and autosomal dominant long QT syndrome (LQTS) worldwide.
Pubmed
24552659
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.585217806841668
Year of publication
2014
Drugs