Explanipedia

Advanced 4-chamber echocardiography techniques enable clinically matched precise characterization of heart disease progression in mice Open

Ege Kacira, Mariam Oueis, Nihad Tamimi, Sarah L. Sturgill, Xianyao Xu , et al. · 2025

The A4CLAX imaging approach provides clinically comparable, comprehensive echocardiographic evaluation in murine models and offers improved sensitivity for detecting subtle changes in cardiac performance during disease progression.

Structural and Functional Mechanisms Underlying Activation Gate Dynamics and IFM Motif Accessibility in Human Nav1.5 Open

Rupam Biswas, Ana Laura López-Serrano, Apoorva Purohit, Angelina Ramirez‐Navarro, Hsiang-Ling Huang , et al. · 2025

Voltage-gated sodium channels are vital for regulating excitability in muscle and nerve cells, and their dysregulation is linked to a range of diseases. However, therapeutic targeting of Na v channels remains challenging due to a limited u…

The Role of Global Longitudinal Strain in Detecting Early Left Ventricular Dysfunction in Pediatric Bicuspid Aortic Valve Patients Open

Mohammed A. Mashali, Isabelle Deschênes, Carl V. Leier, Nancy S. Saad · 2025

Global longitudinal strain (GLS), assessed via speckle tracking echocardiography (STE), is increasingly recognized as a sensitive and early indicator of left ventricular (LV) dysfunction in pediatric patients with bicuspid aortic valve (BA…

Transformative Potential of Induced Pluripotent Stem Cells in Congenital Heart Disease Research and Treatment Open

Mohammed A. Mashali, Isabelle Deschênes, Nancy S. Saad · 2025

Congenital heart disease (CHD), the most common congenital anomaly, remains a significant lifelong burden despite advancements in medical and surgical interventions. Induced pluripotent stem cells (iPSCs) have emerged as a groundbreaking p…

The two-pore K+ channel TREK-1 regulates pressure overload-induced cardiac remodeling Open

C M Johnson, Drew Nassal, Alexander J. Winkle, Benjamin Buck, Xianyao Xu , et al. · 2025

A major finding of this study is the involvement of the background K + channel TREK-1 in modulating STAT3 activation, profibrotic gene expression, and fibrosis with implications for the cardiac remodeling response to chronic pressure overl…

Structural basis of human Na v 1.5 gating mechanisms Open

Rupam Biswas, Ana Laura López-Serrano, Apoorva Purohit, Angelina Ramirez‐Navarro, Hsiang-Ling Huang , et al. · 2025

Voltage-gated Na v 1.5 channels are central to the generation and propagation of cardiac action potentials. Aberrations in their function are associated with a wide spectrum of cardiac diseases including arrhythmias and heart failure. Desp…

473 Application of human induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) for modeling of Ankyrin-2 p.R990Q variant-induced ventricular arrhythmia and personalized medicine Open

Jyotsna Joshi, Neill Schwieterman, Nate Smole, Shuliang Guo, Xiaoping Wan , et al. · 2024

OBJECTIVES/GOALS: The cytoskeletal protein α²II spectrin interacts with actin and ankyrin-2 in cardiomyocytes which is essential to orchestrate ion channels and membrane proteins in the cardiac dyad. Our goal is to understand molecular mec…

Persistent PKA activation redistributes NaV1.5 to the cell surface of adult rat ventricular myocytes Open

Tytus Bernaś, John Seo, Zachary T. Wilson, Bi-hua Tan, Isabelle Deschênes , et al. · 2024

During chronic stress, persistent activation of cAMP-dependent protein kinase (PKA) occurs, which can contribute to protective or maladaptive changes in the heart. We sought to understand the effect of persistent PKA activation on NaV1.5 c…

MicroRNA-1 Deficiency Is a Primary Etiological Factor Disrupting Cardiac Contractility and Electrophysiological Homeostasis Open

Dandan Yang, Xiaoping Wan, Neill Schwieterman, Omer Cavus, Ege Kacira , et al. · 2023

BACKGROUND: MicroRNA-1 (miR1), encoded by the genes miR1-1 and miR1-2 , is the most abundant microRNA in the heart and plays a critical role in heart development and physiology. Dysregulation of miR1 has been associated with various heart …

Long-Axis Biplane Echocardiography Sensitively Detects the Progressing Functional Deterioration of Mouse Heart Open

Ege Kacira, Mitchell Pittman, Dandan Yang, Sarah L. Sturgill, Mark T. Ziolo , et al. · 2023

Biophysical characterization of chloride intracellular channel 6 (CLIC6) Open

Veronica Loyo-Celis, Devendra Patel, Shridhar Sanghvi, Kamalpreet Kaur, Devasena Ponnalagu , et al. · 2023

Chloride intracellular channels (CLICs) are a family of proteins that exist in soluble and transmembrane forms. The newest discovered member of the family CLIC6 is implicated in breast, ovarian, lung gastric, and pancreatic cancers and is …

Extracellular Perinexal Separation Is a Principal Determinant of Cardiac Conduction Open

William P. Adams, Tristan B. Raisch, Yajun Zhao, Rafael V. Davalos, Sarah H. Barrett , et al. · 2023

BACKGROUND: Cardiac conduction is understood to occur through gap junctions. Recent evidence supports ephaptic coupling as another mechanism of electrical communication in the heart. Conduction via gap junctions predicts a direct relations…

Editorial Board Open

Izhak Kehat, Francis Kim, Yibin Wang, Huaxiao Yang, M.-H Zou , et al. · 2023

Editorial Board Open

Izhak Kehat, Jolanda van der Velden, Yibin Wang, Huaxiao Yang, Wolfram‐Hubertus Zimmermann , et al. · 2023

EXTRACELLULAR PERINEXAL SEPARATION IS A PRINCIPAL DETERMINANT OF CARDIAC CONDUCTION Open

William P. Adams, Tristan B. Raisch, Yajun Zhao, Rafael V. Davalos, Sarah H. Barrett , et al. · 2023

Rationale Cardiac conduction is understood to occur through gap junctions. Recent evidence supports ephaptic coupling as another mechanism of electrical communication in heart. Conduction via gap junctions predicts a direct relationship be…

PO-01-123 THE BIOPHYSICAL ACTION OF MICRORNA IS ESSENTIAL TO MAINTAIN THE NORMAL FUNCTION AND ELECTROPHYSIOLOGY OF THE HEART Open

Dandan Yang, Xiaoping Wang, Feng Tang, Ege Kacira, Xianyao Xu , et al. · 2023

PO-01-241 ROLE OF THE TWO-PORE K CHANNEL TREK1 IN REGULATING HEART FAILURE-INDUCED VENTRICULAR ARRHYTHMIA Open

Cemantha Lane, Xianyao Xu, Xiaoping Wan, Isabelle Deschênes, Thomas J. Hund · 2023

Editorial Board Open

Izhak Kehat, Jolanda van der Velden, Yibin Wang, Huaxiao Yang, Wolfram‐Hubertus Zimmermann , et al. · 2023

Precision medicine for long QT syndrome: patient-specific iPSCs take the lead Open

Yang Yu, Isabelle Deschênes, Mingtao Zhao · 2023

Long QT syndrome (LQTS) is a detrimental arrhythmia syndrome mainly caused by dysregulated expression or aberrant function of ion channels. The major clinical symptoms of ventricular arrhythmia, palpitations and syncope vary among LQTS sub…

Impaired Human Cardiac Cell Development due to NOTCH1 Deficiency Open

Shiqiao Ye, Cankun Wang, Zhaohui Xu, Hui Lin, Xiaoping Wan , et al. · 2022

Background: NOTCH1 pathogenic variants are implicated in multiple types of congenital heart defects including hypoplastic left heart syndrome, where the left ventricle is underdeveloped. It is unknown how NOTCH1 regulates human cardiac cel…

Protein 14-3-3 Influences the Response of the Cardiac Sodium Channel Nav1.5 to Antiarrhythmic Drugs Open

Yang Zheng, Isabelle Deschênes · 2022

Editorial Board Open

Jolanda van der Velden, Yibin Wang, Huaxiao Yang, Wolfram‐Hubertus Zimmermann, M.-H Zou , et al. · 2022

Trafficking and Gating Cooperation Between Deficient Nav1.5-mutant Channels to Rescue INa Open

Jérôme Clatot, Alain Coulombe, Isabelle Deschênes, Pascale Guicheney, Nathalie Neyroud · 2022

Background: Pathogenic variants in SCN5A, the gene encoding the cardiac Na+ channel α-subunit Nav1.5, result in life-threatening arrhythmias, e.g., Brugada syndrome, cardiac conduction defects and long QT syndrome. This variety of phenotyp…

Multilayer control of cardiac electrophysiology by microRNAs Open

Dandan Yang, Isabelle Deschênes, Ji‐Dong Fu · 2022

Inhibition of CREB-CBP Signaling Improves Fibroblast Plasticity for Direct Cardiac Reprogramming Open

Emre Bektik, Yu Sun, Adrienne T. Dennis, Phraew Sakon, Dandan Yang , et al. · 2021

Direct cardiac reprogramming of fibroblasts into induced cardiomyocytes (iCMs) is a promising approach but remains a challenge in heart regeneration. Efforts have focused on improving the efficiency by understanding fundamental mechanisms.…

A Heart Failure-Associated SCN5A Splice Variant Leads to a Reduction in Sodium Current Through Coupled-Gating With the Wild-Type Channel Open

Yang Zheng, Xiaoping Wan, Dandan Yang, Angelina Ramirez‐Navarro, Haiyan Liu , et al. · 2021

Na v 1.5, encoded by the gene SCN5A , is the predominant voltage-gated sodium channel expressed in the heart. It initiates the cardiac action potential and thus is crucial for normal heart rhythm and function. Dysfunctions in Na v 1.5 have…

MicroRNA Biophysically Modulates Cardiac Action Potential by Direct Binding to Ion Channel Open

Dandan Yang, Xiaoping Wan, Adrienne T. Dennis, Emre Bektik, Wang Zh , et al. · 2021

Background: MicroRNAs (miRs) play critical roles in regulation of numerous biological events, including cardiac electrophysiology and arrhythmia, through a canonical RNA interference mechanism. It remains unknown whether endogenous miRs mo…

iPSC-derived cardiomyocytes from patients with myotonic dystrophy type 1 have abnormal ion channel functions and slower conduction velocities Open

Hugo Poulin, Aurélie Mercier, Mohammed Djemai, Valérie Pouliot, Isabelle Deschênes , et al. · 2021

Cardiac complications such as electrical abnormalities including conduction delays and arrhythmias are the main cause of death in individuals with Myotonic Dystrophy type 1 (DM1). We developed a disease model using iPSC-derived cardiomyocy…

Long QT syndrome – Bench to bedside Open

Daniela Ponce‐Balbuena, Isabelle Deschênes · 2021

Expression defect of the rare variant/Brugada mutation R1512W depends upon the SCN5A splice variant background and can be rescued by mexiletine and the common polymorphism H558R Open

Rou-Mu Hu, E Song, David J. Tester, Isabelle Deschênes, Michael J. Ackerman , et al. · 2021

Background : Mutations in SCN5A that decrease Na current underlie arrhythmia syndromes such as the Brugada syndrome (BrS). SCN5A in humans has two splice variants, one lacking a glutamine at position 1077 (Q1077del) an…

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