Maturation-Based Model of Arrhythmogenic Right Ventricular Dysplasia Using Patient-Specific Induced Pluripotent Stem Cells

Wen, Jianyan; Wei, Chu; Shah, Khooshbu; Wong, Johnson; Wang, Cheng; Chen, Huei‐Sheng Vincent

doi:10.1253/circj.cj-15-0363

Cited by 49 publications

(40 citation statements)

References 54 publications

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“…3, 44 The findings of the present study show that in the myocardium with non-uniform contraction, Cx43 plays some role in Ca 2+ wave propagation and the occurrence of arrhythmias, probably under the modulation of mitochondrial KATP channels, making Cx43 an attractive therapeutic target for suppressing triggered arrhythmias in such diseased hearts. 45,46 In conclusion, the present study suggests that Cx43 plays an affects ∆Ψm. 39 Some results indicate no effect, and others indicate depolarization of ∆Ψm.…”

Section: Cbx and Ca 2+ Leak From The Srsupporting

confidence: 63%

Effect of Carbenoxolone on Arrhythmogenesis in Rat Ventricular Muscle

Miura

Nagano

Murai

et al. 2016

Circ J

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Section: Cbx and Ca 2+ Leak From The Srsupporting

confidence: 63%

Effect of Carbenoxolone on Arrhythmogenesis in Rat Ventricular Muscle

Miura

Nagano

Murai

et al. 2016

Circ J

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“…Disorders that affect structure, contractility and survival have also been modelled, such as Duchenne muscular dystrophy (DMD) [60], dilated cardiomyopathy [61], [62], hypertrophic cardiomyopathy (HCM) [63], [64], Leopard Syndrome [65], Barth Syndrome [66], [67] and arrhythmogenic right ventricular cardiomyopathy (ARVC) [68], [69], [70], [71]. These have been used to understand disease mechanisms and evaluate novel therapeutics.…”

Section: Introductionmentioning

confidence: 99%

Cardiomyocytes from human pluripotent stem cells: From laboratory curiosity to industrial biomedical platform

Denning

Borgdorff

Crutchley

et al. 2016

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research

247

261

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Cardiomyocytes from human pluripotent stem cells (hPSCs-CMs) could revolutionise biomedicine. Global burden of heart failure will soon reach USD $90bn, while unexpected cardiotoxicity underlies 28% of drug withdrawals. Advances in hPSC isolation, Cas9/CRISPR genome engineering and hPSC-CM differentiation have improved patient care, progressed drugs to clinic and opened a new era in safety pharmacology. Nevertheless, predictive cardiotoxicity using hPSC-CMs contrasts from failure to almost total success. Since this likely relates to cell immaturity, efforts are underway to use biochemical and biophysical cues to improve many of the ~ 30 structural and functional properties of hPSC-CMs towards those seen in adult CMs. Other developments needed for widespread hPSC-CM utility include subtype specification, cost reduction of large scale differentiation and elimination of the phenotyping bottleneck. This review will consider these factors in the evolution of hPSC-CM technologies, as well as their integration into high content industrial platforms that assess structure, mitochondrial function, electrophysiology, calcium transients and contractility. This article is part of a Special Issue entitled: Cardiomyocyte Biology: Integration of Developmental and Environmental Cues in the Heart edited by Marcus Schaub and Hughes Abriel.

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“…Thus, cells other than cardiomyocytes must be involved in the abnormal adipogenesis and fibrosis which is an essential feature of AC phenotype [100]. A role of cardiac mesenchymal stromal cells as a source of adipocytes in AC has been recently suggested [101].…”

Section: Abnormal Intercellular Junction Proteins and Intracellular Smentioning

confidence: 99%