A comparative review on heart ion channels, action potentials and electrocardiogram in rodents and human: extrapolation of experimental insights to clinic

Joukar, Siyavash

doi:10.1186/s42826-021-00102-3

Laboratory Animal Research

Table 5 The genetic basis of some of inherited arrhythmias along with existing animals’ transgenic models

From: A comparative review on heart ion channels, action potentials and electrocardiogram in rodents and human: extrapolation of experimental insights to clinic

Arrhythmia syndrome	Current	Channel/protein	Gen	Functional Alteration	Reported Models	Ref
Familial atrial fibrillation	IKs	Potassium channel (α-subunit)	KCNQ1	Gain of function	Mouse	[9, 76, 82, 83]
	IKr	Potassium channel β-subunit	KCNE2	Gain of function	Mouse	[9, 76, 82, 83]
	IKr	Potassium channel α-subunit	KCNH2	Gain of function		[9, 82, 83]
	IK1	Potassium channel subunit	KCNJ2	Gain of function		[9, 82, 83]
	IKUr	Potassium channel subunit	KCNA5	Loss of function		[81,82,83]
	INa	Sodium channel α-subunit	SCN5A	Loss of function		[81,82,83]
Cardiac conduction disease	INa	Sodium channel α-subunit	SCN5A	Loss of function	Mouse	[9, 73]
Sick sinus syndrome	If	Pacemaker channel subunit	HCN4	Loss of function	Mouse	[7, 9, 50, 80]
Sick sinus syndrome	INa	sodium channel α-subunit	SCN5A	Loss of function		[9, 80]
Brugada syndromes
BrS1	INa	Sodium channel α-subunit	SCN5A	Loss of function	Mouse, Porcine, Canine	[7, 9, 74, 80, 82]
BrS2	Regulation of INa	Glycerol-3-phosphate dehydrogenase 1-like	GPD1L	Altered function		[7, 9, 80, 82]
BrS3	ICa	calcium channel α-subunit	CACNA1C	Loss of function		[7, 9, 78, 80, 82]
BrS4	ICa	Calcium channel β-subunit	CACNB2b	Loss of function		[7, 9, 78, 80, 82]
Long QT syndromes
LQTS1	IKs	Potassium channel α-subunit	KCNQ1	Loss of function	Rabbit, mouse	[7, 9, 70, 71, 79, 80, 82]
LQTS2	IKr	Potassium channel α-subunit	KCNH2	Loss of function	Rabbit, Mouse	[7, 9, 70, 71, 79, 80, 82]
LQTS3	INa	Sodium channel α-subunit	SCN5A	Gain of function	Mouse	[7, 9, 79, 80, 82]
LQTS5	IKs	Potassium channel β-subunit	KCNE1	Loss of function	Rabbit, Mouse	[7, 9, 70, 75, 79, 82]
LQTS6	IKr	Potassium channel β-subunit	KCNE2	Loss of function		[7, 9, 79, 80]
LQTS7	IK1	Potassium channel subunit	KCNJ2	Loss of function	Mouse	[7, 9, 79, 80, 82]
LQTS8	ICa	Calcium channel α-subunit	CACNA1C	Gain of function		[7, 9, 78,79,80, 82]
Short QT syndrome
SQTS1	IKr	Potassium channel α-subunit	KCNH2	Gain of function	Rabbit	[7, 9, 72, 77, 80, 82]
SQTS2	IKs	potassium channel α-subunit	KCNQ1	Gain of function		[7, 9, 77, 80, 82]
SQTS3	IK1	Potassium channel subunit	KCNJ2	Gain of function		[7, 9, 77, 80, 82]
SQTS4	ICa	Calcium channel α-subunit	CACNA1C	Loss of function		[77, 80]
SQTS5	ICa	Calcium channel β2-subunit	CACN2B	Loss of function		[77, 80]
SQTS6	ICa	Calcium channel α2δ1-subunit	CACNA2D1	Loss of function		[77, 80, 82]

LQTS, long QT syndrome; SQTS, short QT syndrome; BrS, Brugada syndrome; INa, Fast Na⁺ current; IKur, ultra-rapid outward K⁺ current; ICa, Ca²⁺ current; IKr, Rapid delayed rectifier K⁺ current; IKs, Slow delayed rectifier K⁺ current; IK1, inward rectifier potassium current

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ISSN: 2233-7660

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