Paralogue Annotation for KCNH2 residue 1007

Residue details

Gene: KCNH2
Reference Sequences: LRG: LRG_288, Ensembl variant: ENST00000262186 / ENSP00000262186
Amino Acid Position: 1007
Reference Amino Acid: R - Arginine
Protein Domain: C-terminus


Paralogue Variants mapped to KCNH2 residue 1007

No paralogue variants have been mapped to residue 1007 for KCNH2.



KCNH2-R----G----------------------->R<QYQELPRCPAPTP-SLLN----IPL-SSPG1031
KCNH1-G---------------------------->-<--KSEDWNKVSKA-ESME----TLP-E---864
KCNH3-Q---------------------------->P<KFSFRVGQSGPE--CSSSPSPGPES-G---861
KCNH4-E----A----------------------->P<SFRFSRRPELPRP-RSQA----PPT-GTRP865
KCNH5-G----A----------------------->H<EEKKEDWNNVTKA-ESMG----LLS-EDPK845
KCNH6-M----P----------------------->Q<GHASYILEAPASN-DLAL----VPI-ASET881
KCNH7-S----P----------------------->S<ALQRAAWGISET----------------ES1024
KCNH8-E----S----------------------->Q<TFDFGSERIRSEP-RISP-----PLGDPEI851
CNGA1------------------------------>-<----------T-------------------690
CNGA2------------------------------>-<----------P-------------------664
CNGA3------------------------------>-<----------Q-------------------694
CNGA4------------------------------>G<RASQEGPPGPE-------------------575
CNGB1------------------------------>P<RTPPEPP---------G------SP-PSSP1200
CNGB3------------------------------>G<REPEEKPLDRPECTA-S------PI-AVEE765
HCN1----------------QQ------------>-<VQQSQPPQTQPQQPS-PQPQTPG---S--S761
HCN2----------------A-P----------->-<-ASPRAPR-----TS--------------P794
HCN3----------------PP----------AR>T<LHASLSRAGRSQ-VSLLG--PP--------697
HCN4VAGASGGASPVGFTPRGGLSPPGHSPGPPR>T<FPSAPPRASGSH-GSLLL--PPASS-P--P1061
cons                              > <                              

See full Alignment of Paralogues


Known Variants in KCNH2

ProteinCDSDisease ClassificationDiseasedbSNP linksEffect Prediction
p.R1007Hc.3020G>A Inherited ArrhythmiaLQTSSIFT: tolerated
Polyphen: benign
ReportsInherited ArrhythmiaLQTS Spectrum and prevalence of mutations from the first 2,500 consecutive unrelated patients referred for the FAMILION long QT syndrome genetic test. Heart Rhythm. 2009 6(9):1297-303. 19716085
Inherited ArrhythmiaLQTS End-recovery QTc: a useful metric for assessing genetic variants of unknown significance in long-QT syndrome. J Cardiovasc Electrophysiol. 2012 23(6):637-42. doi: 10.1111/j.1540-8167.2011.02265. 22429796
Inherited ArrhythmiaLQTS Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome. Nat Commun. 2014 5:5535. doi: 10.1038/ncomms6535. 25417810