Table 2.

Identified genes and modifier genes linked to Brugada syndrome.

BrS subtype	Ion channel	BrS-associated Gene	Locus	Protein	Functional Effect	% of Disease	Reference
BrS1	Sodium channel	SCN5A	3p21	Nav1.5	LOF	20–25 (Caucasian); 10-15 (Asian)	[8], [17]
BrS5		SCN1B	19q13.1	Navβ1	LOF	1–2	[25], [38]
BrS17		SCN2B	11q23	Navβ2	LOF	Rare	[26]
BrS7		SCN3B	11q23.2	Navβ3	LOF	Rare	[27]
BrS18		SCN10A	3p22.2	Nav1.8	LOF	2.5–16	[28], [29], [30], [31], [46]
BrS11	Sodium channel-associated	RANGRF	17p13.1	MOG1	LOF	Rare	[32]
BrS2		GPD1-L	3p24	G3PD1L	LOF	Rare	[33], [38]
BrS15		SLMAP	3p21.2-p14.3	SLMAP	LOF	Rare	[34]
BrS20		PKP2	12p11.21	plakophilin-2	plakophilin-2 cause sodium current deficit	2.5	[35]
BrS16		TRPM4	19q13.33	NSCCa	LOF	8	[36]
BrS3	Calcium channel	CACNA1C	12p13.3	Cav1.2	LOF	6–7	[37], [38]
BrS4		CACNB2b	10p12.33	Cavβ2	LOF	4–5	[37], [38]
BrS10		CACNA2D1	7q21-22	Cavα2δ−1	LOF	Rare	[39]
BrS21	Potassium channel	ABCC9	12p12.1	SUR2A (sulfonylurea receptor subunit 2 A), IK-ATP	GOF	4–5	[44]
BrS13		KCND3	1p13.2	Kv4.3, Ito	GOF	Rare	[40]
BrS6		KCNE3	11q13-14	MiRP2, Ito/Iks	GOF	<1	[38], [41]
BrS9		KCNJ8	12p12.1	Kir6.1, IK-ATP	GOF	Rare	[43], [69]
BrS8		KCNH2	7q35	Kv11.1, Ikr	GOF	1–2	[45]
		BrS-modifier Gene
BrS19	Sodium channel	HEY2	6q22	Nav1.5	LOF	?	[46]
BrS14	Potassium channel	HCN4	15q24.1	If	LOF	Rare	[47], [70]
BrS12		KCNE5	Xq22.3	MiRP4, Kv4.3, Ito	GOF	Rare	[42]