Table 1

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

GeneProteinLevel of evidence
AKAP9 A kinase anchor protein 9Disputed
ANK2 Ankyrin-2Disputed
CACNA1C Calcium voltage-gated channel α1c subunitModerate
CALM1 Calmodulin-1Definitive
CALM2 Calmodulin-2Definitive
CALM3 Calmodulin-3Definitive
CAV3 Caveolin-3Limited
KCNE1 Potassium voltage-gated channel subfamily E regulatory subunit 1Disputed
KCNE2 Potassium voltage-gated channel subfamily E regulatory subunit 1Disputed
KCNH2 Potassium voltage-gated channel subfamily H member 2Definitive
KCNJ2 Potassium voltage-gated channel subfamily J member 2Limited
KCNJ5 Potassium voltage-gated channel subfamily J member 5Disputed
KCNQ1 Potassium voltage-gated channel subfamily Q member 2Definitive
SCN4B Sodium voltage-gated channel β subunit 4Disputed
SCN5A Sodium channel voltage-gated α subunit 5Definitive
SNTA1 Syntrophin α1Disputed
TRDN TriadinStrong

  • *See for more details ref 11.

  • LQTs, long QT syndrome .