Altered contractility, Ca<sup>2+</sup> transients, and cell morphology seen in a patient-specific iPSC-CM model of Ebstein’s anomaly with left ventricular noncompaction-Reference-Cited by-同舟云学术

Altered contractility, Ca²⁺ transients, and cell morphology seen in a patient-specific iPSC-CM model of Ebstein’s anomaly with left ventricular noncompaction

Published:2023-07-01 Issue:1 Volume:325 Page:H149-H162
ISSN:0363-6135
Container-title:American Journal of Physiology-Heart and Circulatory Physiology
language:en
Short-container-title:American Journal of Physiology-Heart and Circulatory Physiology

Author:

Thareja Suma K.¹²^ORCID,Anfinson Melissa¹²,Cavanaugh Matthew³,Kim Min-Su¹²,Lamberton Peter³,Radandt Jackson³,Brown Ryan⁴,Liang Huan-Ling¹,Stamm Karl¹^ORCID,Afzal Muhammad Zeeshan²,Strande Jennifer²^ORCID,Frommelt Michele A.⁵⁶,Lough John W.²,Fitts Robert H.³,Mitchell Michael E.¹⁶,Tomita-Mitchell Aoy¹⁶^ORCID

Affiliation:

1. Division of Congenital Heart Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, United States

2. Department of Cell Biology, Neurobiology, and Anatomy, Medical College of Wisconsin, Milwaukee, Wisconsin, United States

3. Department of Biological Sciences, Marquette University, Milwaukee, Wisconsin, United States

4. Department of Microbiology and Immunology, Medical College of Wisconsin, Milwaukee, Wisconsin, United States

5. Division of Pediatric Cardiology, Department of Pediatrics, Children’s Wisconsin, Milwaukee, Wisconsin, United States

6. Herma Heart Institute, Children’s Wisconsin, Milwaukee, Wisconsin, United States

Abstract

We demonstrate here that iPSCs derived from patients with Ebstein’s anomaly and left ventricular noncompaction, when differentiated into cardiomyocytes, display significant structural and functional changes that offer insight into disease pathogenesis, including altered ER/SR and mitochondrial morphology, contractility, and calcium signaling.

Funder

Medical College of Wisconsin

American Heart Association

Children's Hospital of Wisconsin Research Institute

HHS | NIH | National Institute of General Medical Sciences

MCW | Cardiovascular Center, Medical College of Wisconsin

Publisher

American Physiological Society

Subject

Physiology (medical),Cardiology and Cardiovascular Medicine,Physiology

Link

https://journals.physiology.org/doi/pdf/10.1152/ajpheart.00658.2022

Reference88 articles.

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