Table 1

Classification of genetic evidence by the Clinical Genome Resource (ClinGen) for genes previously associated with LQTS

GeneProteinLevel of evidence
AKAP9A kinase anchor protein 9Disputed
ANK2Ankyrin-2Disputed
CACNA1CCalcium voltage-gated channel α1c subunitModerate
CALM1Calmodulin-1Definitive
CALM2Calmodulin-2Definitive
CALM3Calmodulin-3Definitive
CAV3Caveolin-3Limited
KCNE1Potassium voltage-gated channel subfamily E regulatory subunit 1Disputed
KCNE2Potassium voltage-gated channel subfamily E regulatory subunit 1Disputed
KCNH2Potassium voltage-gated channel subfamily H member 2Definitive
KCNJ2Potassium voltage-gated channel subfamily J member 2Limited
KCNJ5Potassium voltage-gated channel subfamily J member 5Disputed
KCNQ1Potassium voltage-gated channel subfamily Q member 2Definitive
SCN4BSodium voltage-gated channel β subunit 4Disputed
SCN5ASodium channel voltage-gated α subunit 5Definitive
SNTA1Syntrophin α1Disputed
TRDNTriadinStrong

  • *See for more details ref 11.

  • LQTs, long QT syndrome .