Modelling Human Channelopathies Using Induced Pluripotent Stem Cells: A Comprehensive Review

Table 1

Current iPS cell-based models for long QT syndromes.

LQTS subtype	Gene mutation	Protein	iPSC-C model
LQTS1	KCNQ1	Alpha-subunit of the delayed rectifier (slow) potassium channel (𝐼ks)	Moretti et al., 2010 [25]
LQTS2	HERG	Alpha-subunit of the delayed rectifier (rapid) potassium channel (𝐼kr)	Itzhaki et al., 2011 [15], Matsa et al., 2011 [26], Lahti et al., 2012 [27]
LQTS3	SNC5A	Alpha-subunit of the cardiac sodium channel	Malan et al., 2011 [28], Davis et al., 2012 [17]
LQTS8	CACNA1c	Alpha-1c-subunit of the L-type calcium channel	Yazawa et al., 2011 [20]