Table 1.

studies of channelopathies using pediatric patient-derived iPSC-CMs.

PMID	Journal	Year	First Author	Disease	Phenotype	Patient description
20660394 ²⁵	N Engl J Med	2010	Alessandr a Moretti	Long-QT Syndrome type 1	Prolonged action potential, reduction in Ik current, altered channel activation	8-year-old boy with ADHD, R190Q in KCNQ1
22739119 ²⁶	Cardiovas c Res	2012	Toru Egashira	Long-QT syndrome	Downregulation of KCNQ1 at peripheral cite, smaller IKs peak and tail current	13-year-old boy, heterozygous deletion of KCNQ1, 1893delC
23277474 ²⁷	J Gen Physiol	2013	Cecile Terrenoire	Long-QT syndrome type 3	Dysfunctional inactivation of Na channel activity	4-year-old, SCN5A F1473Cmutation, KCNH2_K8 97T mutation
23998552 ²⁸	Int J Cardio	2013	Dongrui Ma	Long-QT syndrome type 3	Prolonged action potential duration or APD, increased TTX-sensitive late or persistent Na current	7-year-old Chinese girl, SCN5A V1763M mutation
28956012 ²⁹	Biochem Biophys Rep	2017	Yusuke Kuroda	Andersen-Tawil syndrome	Strong arrhythmic events, higher incidence of irregular Ca release	10-year-old male with a KCNJ2 R218W mutation
28335032 ³⁰	Hum Mol Genet	2017	Yuta Yamamoto	Long-QT syndrome type 15	Significantly lower beating rates, prolonged duration, impaired inactivation of LTCC currents	A 12-year- old boy with LQTS carrying a heterozygou s CALM2 N98S mutation
28158429 ³¹	Cardiovasc Res	2017	Marcella Rocchetti	Long-QT syndrome	CALM-F142L prolonged repolarization, altered its rate-dependency and its response to isoproterenol	A 14-year- old male with CALM1-F142L mutation
22050625 ³²	J Cell Mol Med	2012	Atara Novak	Catechola minergic polymorphic ventricular tachycardia	Isoproterenol causes delayed afterdepolarizat ions, oscillary arrhythmic prepotentials, aftercontractions and diastolic Ca rises	A 12-year- old boy and a 30-year- old woman with CASQ2 D307H mutation
25453094 ³³	Proc Natl Acad Sci U S A	2014	Miao Zhang	Jervell & Lange-Nielsen syndrome	Pronounced action and field potential prolongation and reduction or absence of Iks	Three pediatric patients with KCNQ1 c.478–2A>T and c.1781G>A
21367833 ³⁴	Eur Heart J	2011	Elena Matsa	Long-iPSCQT syndrome type 2	Prolonged field/action potential duration, developed early after depolarization when challenged with isoprenaline	15-year-old LQT2 patient with KCNH2 G1681A mutation