Patient-specific induced pluripotent stem cells implicate intrinsic impaired contractility in hypoplastic left heart syndrome

SL Paige, FX Galdos, S Lee, ET Chin, S Ranjbarvaziri… - Circulation, 2020 - Am Heart Assoc
SL Paige, FX Galdos, S Lee, ET Chin, S Ranjbarvaziri, DAM Feyen, AK Darsha, S Xu…
Circulation, 2020Am Heart Assoc
A, Induced pluripotent stem cells (iPSCs) were derived from patients with hypoplastic left
heart syndrome (HLHS) and controls. IPSCs were differentiated into cardiomyocytes (iPSC-
CMs) using standard Wnt pathway modulation protocols. On day 30 of differentiation, iPSC-
CMs were subjected to assays of contractility, RNA sequencing (RNA-seq), and
mitochondrial function. B, Cardiac troponin T (cTnT) expression in control and HLHS day 30
iPSC-CMs was measured by using flow cytometry. No significant difference was identified in …
A, Induced pluripotent stem cells (iPSCs) were derived from patients with hypoplastic left heart syndrome (HLHS) and controls. IPSCs were differentiated into cardiomyocytes (iPSC-CMs) using standard Wnt pathway modulation protocols. On day 30 of differentiation, iPSC-CMs were subjected to assays of contractility, RNA sequencing (RNA-seq), and mitochondrial function. B, Cardiac troponin T (cTnT) expression in control and HLHS day 30 iPSC-CMs was measured by using flow cytometry. No significant difference was identified in cardiac differentiation efficiency comparing pooled iPSC lines from control individuals (n= 5 lines, 6 biological replicates) with those from 3 HLHS individuals (HLHS1, HLHS2, HLHS3, n= 12 biological replicates each).(Continued)
Am Heart Assoc