Annotation Detail

Information
Associated Genes
KCNQ1
Associated Variants
KCNQ1 p.Arg397Gly (p.R397G) ( ENST00000713725.1, ENST00000646564.2, ENST00000335475.6, ENST00000496887.7, ENST00000155840.12 )
KCNQ1 p.Arg397Trp (p.R397W) ( ENST00000155840.12, ENST00000496887.7, ENST00000646564.2, ENST00000335475.6, ENST00000713725.1 )
KCNQ1 p.Arg397Gly (p.R397G) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Arg397Trp (p.R397W) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
Associated Disease
long QT syndrome
Source Database
DisGeNET
Description
Both KV7.1-A283T (16-week male) and KV7.1-R397W (16-week female) mutations were associated with marked KV7.1 loss-of-function consistent with in utero LQTS type 1, whereas the HERG1b-R25W mutation (33.2-week male) exhibited a loss of function consistent with in utero LQTS type 2.
Pubmed
23571586
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.41651083741347
Year of publication
2013
Drugs