Table 2.
Congenital long-QT syndrome (LQTS)
| Name | Current | Gain/loss of function | Protein | Gene | Reference |
|---|---|---|---|---|---|
| LQT1 | IKs | Loss | KvLQT1 | KCNQ1 | Wang et al3 |
| LQT2 | IKr | Loss | Kv11.1 | KCNH2 | Curran et al2 |
| LQT3 | INa | Gain | Nav1.5 | SCN5A | Wang et al4 |
| LQT4 | INCX, INaK, InsP3R | Loss | Ankyrin-B | ANKB | Mohler et al9 |
| LQT5 | IKs | Loss | MinK | KCNE1 | Splawski et al93 |
| LQT6 | IKr | Loss | MiRP1 | KCNE2 | Abbott et al94 |
| LQT7 | IK1 | Loss | Kir2.1 | KCNJ2 | Plaster et al,16 Ai et al95 |
| LQT8 | ICa,L | Gain | Cav1.2α1 | CACNA1C | Splawski et al25 |
| LQT9 | INa | Gain | Caveolin-3 | CAV3 | Vatta et al,10 Ye et al96 |
| LQT10 | INa | Gain | Navβ4 | SCN4B | Domingo et al,97 Medeiros-Domingo et al98 |
| LQT11 | IKs | Loss | Yotiao | AKAP9 | Chen et al13 |
| LQT12 | INa | Gain | α-1 Syntrophin | SNTA1 | Ueda et al,99 Wu et al100 |
| LQT13 | IK-Ach | Loss | Kir3.4 | KCNJ5 | Yang et al101 |
Abbreviations: IKr and IKs, rapid and slow delayed-rectifier potassium currents respectively; INa, sodium current; INCX, Na+/Ca2+ exchanger current; INaK, Na+/K+ ATPase (pump) current; InsP3R, inositol 1,4,5-trisphosphate (IP3) receptors; IK1, inward-rectifier potassium current; ICa,L, L-type calcium current; IK-Ach, acetylcholine-regulated potassium current.