Table 3.
SQTS Subtype | Gene | Channel Function | Mechanism |
---|---|---|---|
SQTS1 | KCNH2 | α-subunit IKr | Gain-of-function |
SQTS 2 | KCNH2 | α-subunit IKs | Gain-of-function |
SQTS 3 | KCNJ2 | α-subunit IK1 | Gain-of-function |
SQTS 4 | CACNA1C | α-subunit ICaL | Loss-of-function |
SQTS 5 | CACNB2 | β2-subunit ICaL | Loss-of-function |
SQTS 6 | CACNA2D1 | Δ1-subunit ICaL | Loss-of-function |
SQTS 7 | SCN5A | α-subunit INa | Loss-of-function |
SQTS 8 | SLC4A3 | AE3 anion exchanger | Loss-of-function |
SQTS—short QT syndrome.