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. 2015 Jun 25;38(10):855–867. doi: 10.1007/s40264-015-0316-6

Table 1.

Genes known from congenital long QT syndrome, investigated in drug-induced long QT syndrome or torsade de pointes

cLQTS type Gene abbreviation Gene name [110] Gene protein product [9] Association founda
Yes No
cLQTS1 KCNQ1 Potassium channel, voltage gated KQT-like subfamily Q, member 1 gene IKs channel alpha-subunit 3 4
cLQTS2 KCNH2 Potassium channel, voltage gated eag-related subfamily H, member 2 IKr channel alpha-subunit 9 4
cLQTS3 SCN5A Sodium channel, voltage gated, type V alpha-subunit INa channel alpha-subunit 3 4
cLQTS4 ANK2 Ankyrin 2, neuronal 1 1
cLQTS5 KCNE1 Potassium channel, voltage gated subfamily E regulatory beta-subunit 1 IKs channel beta-subunit 4 3
cLQTS6 KCNE2 Potassium channel, voltage gated subfamily E regulatory beta-subunit 2 IKr channel beta-subunit 2 6
cLQTS7 KCNJ2 Potassium channel, inwardly rectifying subfamily J, member 2 IKl alpha-subunit 0 3
cLQTS8 CACNA1C Calcium channel, voltage-dependent, L type, alpha-1C subunit ICaL channel alpha-subunit 2 1
cLQTS9 CAV3 Caveolin-3 0 2
cLQTS10 SCN4B Sodium channel, voltage gated, type IV beta-subunit INa channel beta-subunit 4 1 1
cLQTS11 AKAP9 A kinase anchor protein 9 1 2
cLQTS12 SNTA1 Syntrophin, acidic 1 1 1
cLQTS13 KCNJ5 Potassium channel, inwardly rectifying subfamily J, member 5 IKACh channel alpha-subunit 0 1

cLQTS congenital long QT syndrome

aNumber of studies that did or did not find an association with the corresponding gene