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Table 2 Patient-specific iPSC-CMs in cardiac disease modeling

From: The march of pluripotent stem cells in cardiovascular regenerative medicine

Disease modeled Genetic disorder Phenotypical assessment iPSC-CM abnormality Patients Control Reference(s)
LQTS-1 KCNQ1 Patch clamp
Immunohistochemistry
Iks decrease
Adrenergic response
2 2 healthy individuals [10]
LQTS-2 KCNH2 Patch clamp
Electron recording
Pharmacology
Iks decrease
APD prolongation
1 1 healthy individual [229]
LQTS-2 KCNH2 Patch clamp
Microscopy
APD prolongation
Drug sensitivity increase
2 CMs from HUES7 cell lines and genetically unrelated hESC-derived fibroblasts [13]
LQTS-8 (Timothy syndrome) CACNA1C Patch clamp ICa 2 2 healthy individuals [11]
Leopard syndrome (HCM) PTPN11 Microscopy
Immunocytochemistry
Western blotting
Antibody array
Large cells, high degree of sarcomeric organization, preferential nuclear localization of NFATC4 2 hESCs and 1 healthy individual [12]
DCM TNNT2 Patch clamp
Electrode recordings
Microelectrode array
Atomic force microscopy
Altered Ca2+ handling
Decreased contractility, abnormal sarcomeric organization, increased susceptibility to adrenergic stimulation and bio-mechanical stress
Many Many healthy individuals [257]
  1. ADP action potential duration, DCM dilated cardiomyopathy, hESC human embryonic stem cell, iPSC-CM induced pluripotent stem cell-derived cardiomyocyte, Ikr delayed rectifier potassium current, Iks slow outward potassium current, LQTS long QT syndrome