Table 7.

Gene defects associated with the early repolarization syndrome (ERS) and Brugada (BrS) syndrome.

Genetic Defects Associated with ERS
	Locus	Gene/protein	Ion channel	Percent of Probands
ERS1	12p11.23	KCNJ8, Kir6.1	↑ IK-ATP	Rare
ERS2	12p13.3	CACNA1C, Cav1.2	↓ ICa	4.1%
ERS3	10p12.33	CACNB2b, Cavß2b	↓ ICa	8.3%
ERS4	7q21.11	CACNA2D1, Cavα2δ1	↓ ICa	4.1%
ERS5	12p12.1	ABCC9, SUR2A	↑ IK-ATP	Rare
ERS6	3p21	SCN5A, Nav1.5	↓ INa	Rare
ERS7	3p22.2	SCN10A, Nav1.8	↓ INa	Rare
Genetic Defects Associated with BrS
	Locus	Gene/protein	Ion channel	Percent of probands
BrS1	3p21	SCN5A, Nav1.5	↓ INa	11–28%
BrS2	3p24	GPD1L	↓ INa	Rare
BrS3	12p13.3	CACNA1C, Cav1.2	↓ Ca	6.6%
BrS4	10p12.33	CACNB2b, Cavß2b	↓ ICa	4.8%
BrS5	19q13.1	SCN1B, Navß1	↓ INa	1.1%
BrS6	11q13-14	KCNE3, MiRP2	↑ Ito	Rare
BrS7	11q23.3	SCN3B, Navß3	↓ INa	Rare
BrS8	12p11.23	KCNJ8, Kir6.1	↑ IK-ATP	2%
BrS9	7q21.11	CACNA2D1, Cav α2δ1	↓ ICa	1.8%
BrS10	1p13.2	KCND3, Kv4.3	↑ Ito	Rare
BrS11	17p13.1	RANGRF, MOG1	↓ INa	Rare
BrS12	3p21.2-p14.3	SLMAP	↓ INa	Rare
BrS13	12p12.1	ABCC9, SUR2A	↑ IK-ATP	Rare
BrS14	11q23	SCN2B, Navß2	↓ INa	Rare
BrS15	12p11	PKP2, Plakophillin-2	↓ INa	Rare
BrS16	3q28	FGF12, FHAF1	↓ INa	Rare
BrS17	3p22.2	SCN10A, Nav1.8	↓ INa	5–16.7%
BrS18	6q	HEY2 (transcriptional factor)	↑ INa	Rare

Listed in chronologic order of their discovery.