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. Author manuscript; available in PMC: 2009 Jan 1.
Published in final edited form as: Cardiol J. 2008;15(2):100–121.

Table 1.

Inherited disorders caused by ion channelopathies

Rhythm Inheritance Locus Ion channel Gene
Long QT syndrome (RW) TdP AD
LQT1 11p15 IKs KCNQ1, KvLQT1
LQT2 7q35 IKr KCNH2, HERG
LQT3 3p21 INa SCN5A, Nav1.5
LQT4 4q25 ANKB, ANK2
LQT5 21q22 IKs KCNE1, minK
LQT6 21q22 IKr KCNE2, MiRP1
LQT7 (Andersen-Tawil syndrome) 17q23 IK1 KCNJ2, Kir 2.1
LQT8 (Timothy syndrome) 6q8A ICa CACNA1C, Cav1.2
LQT9 3p25 INa CAV3, Caveolin-3
LQT10 11q23.3 INa SCN4B. Navb4
LQT syndrome (JLN) TdP AR 11p15 IKs KCNQ1, KvLQT1
21q22 IKs KCNE1, minK
Brugada syndrome BrS1 PVT AD 3p21 INa SCN5A, Nav1.5
BrS2 PVT AD 3p24 INa GPD1L
BrS3 PVT AD 12p13.3 ICa CACNA1C, CaV1.2
BrS4 PVT AD 10p12.33 ICa CACNB2β, Cavβ2b

AD — autosomal dominant; AR — autosomal recessive; JLN — Jervell and Lange-Nielsen; LQT — long QT; RW — Romano-Ward; TdP — torsade de pointes; VF — ventricular fibrillation; VT — ventricular tachycardia; PVT — polymorphic VT