Annotation Detail

Information
Associated Genes
KCNH2
Associated Variants
KCNQ1 p.Pro448Gln (p.P448Q) ( ENST00000713725.1, ENST00000335475.6, ENST00000646564.2, ENST00000496887.7, ENST00000155840.12 )
KCNQ1 p.Pro448Arg (p.P448R) ( ENST00000335475.6, ENST00000155840.12, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Pro448Leu (p.P448L) ( ENST00000496887.7, ENST00000155840.12, ENST00000646564.2, ENST00000335475.6, ENST00000713725.1 )
KCNQ1 p.Pro448Gln (p.P448Q) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Pro448Arg (p.P448R) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
KCNQ1 p.Pro448Leu (p.P448L) ( ENST00000155840.12, ENST00000335475.6, ENST00000496887.7, ENST00000646564.2, ENST00000713725.1 )
Associated Disease
Congenital long QT syndrome
Source Database
DisGeNET
Description
Given the inconsistencies between the genotype (LQT1) and clinical phenotype (LQT2) in our two LQTS families, together with the finding that the P448R appears to be a common, ethnic-specific polymorphism, mutational analysis was extended to the other LQTS-causing genes resulting in the identification of distinct HERG missense mutations in each of these two families.
Pubmed
15242738
Original source reporting the Gene Disease association
BeFree
DisGENET score for the Gene Disease association
0.131672000499453
Year of publication
2004
Drugs