Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes Afford New Opportunities in Inherited Cardiovascular Disease Modeling

Table 3

Human IPSC-derived patient-specific LQT syndrome cell models.

Syndrome type	Gene	Protein	Mutation	Donor cell types	Reprogramming method	References
LQT1	KCNQ1	Potassium voltage-gated channel subfamily KQT member 1	p.R190Q	Fibroblasts	RV, OSKM	[24]
			1893delC (P631fs/33)	Fibroblasts	LV, OSKM	[25]
LQT2	KCNH2	Potassium voltage-gated channel subfamily H member 2	p.A614V	Fibroblasts	RV, OSK	[26]
			p.A561T	Fibroblasts	LV, ONSL	[27]
			p.R176W	Fibroblasts	RV, OSKM	[29]
			p.G603D	T-lymphocytes	SV, OSKM	[92]
			p.N996I	Fibroblasts	RV, OSKM	[35]
LQT3	SCN5A	Sodium channel protein type 5 subunit alpha	p.F1473C	Fibroblasts	RV, OSKM	[30]
			p.V1763M	Fibroblasts	mRNA, OSKM	[31]
LQT4	ANK2	Ankyrin-2
LQT5	KCNE1	Potassium voltage-gated channel subfamily E member 1
LQT6	KCNE2	Potassium voltage-gated channel subfamily E member 2
LQT7	KCNJ2	Inward rectifier potassium channel 2
LQT8	CACNA1	Voltage-dependent P-type/Q-type calcium channel subunit alpha-1A	p.G406R	Fibroblasts	RV, OSKM	[5]
LQT9	CAV3	Caveolin-3
LQT10	SCN4B	Sodium channel subunit beta-4 precursor
LQT11	AKAP9	A-kinase anchor protein 9
LQT12	SNTA1	Alpha-1-syntrophin
LQT13	KCNJ5	G protein-activated inward rectifier potassium channel 4

RV: retroviruses, LV: lentiviruses, SV: sendai virus, and OCT4 (O), SOX2 (S), KLF4 (K), c-MYC (M), NANOG (N), and LIN28 (L). Ion channels: : slow delayed rectifier K+ current; : rapid delayed rectifier K+ current; : sodium channel current; : inwardly rectifying K+ current; : L-type calcium current; and : acetylcholine activated potassium current.