About this Research Topic
Inherited cardiac arrhythmias are syndromes associated with potentially life-threatening ventricular arrhythmias and sudden cardiac death, especially in the young. They are caused by genetic mutations of ion channels and related regulatory proteins that alter cardiac cell membrane potential and intracellular homeostasis. Long QT syndromes, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, short QT syndrome are the main inherited cardiac arrhythmias.
The pathophysiological mechanisms of inherited cardiac arrhythmias syndromes have been investigated using experimental model systems, including transgenic murine models, canine heart preparations, zebrafish, and expression systems, to study different gene mutations. The recent development of induced pluripotent stem cell (iPS)-derived cardiomyocytes creates an opportunity to study cardiomyocytes derived from patients and healthy individuals. This Research Topic focuses on basic electrophysiological research using experimental models for unravelling the specific mechanisms, the action of modifier genes, and exploring novel therapeutic strategies.
Genetically modified murine models have allowed for detailed investigation of the in vivo, ex vivo, and in vitro electrophysiological consequences of human mutations associated with inherited cardiac arrhythmias syndromes. However, certain cardiac electrophysiological properties are inherently different between mouse and human. Perfused wedges of canine left or right ventricles rather than a whole heart have been widely used to study cardiac electrophysiology. Zebrafish are relatively easy to study and genetically modify. Ion channel disorders related to repolarization disorders have been successfully modelled in zebrafish. More recently, human-induced pluripotent stem cell-derived (iPSC) cardiomyocytes may recapitulate disease phenotypes in inherited cardiac rhythm disorders. Due to iPSC-CM relative immature phenotypes, caution should be taken when interpreting electrophysiological data from iPSC-derived cardiomyocytes. Nevertheless, it is a promising tool for studying pathophysiology, genotype-phenotype relationship, and pharmacology in cardiac arrhythmia syndromes.
In the last few decades, significant progress has been made in clinical practice and basic research to understand the natural history and mechanisms of the inherited cardiac arrhythmias syndromes. However, those highly potentially life-threatening ventricular arrhythmias and sudden cardiac death, especially in young, still largely remain unexplored. This Research Topic aims to investigate and better understand the mechanisms of causing genes, the action of modifier genes and explore innovation therapy strategies using experiment models. A deeper knowledge of these mechanisms may help in further elucidating the pathophysiology and explore innovation therapy strategies.
This Research Topic focuses on the electrophysiology of the inherited cardiac arrhythmias syndromes, such as LQT, CPVT, Brugada syndrome, SQT, and conduction disorders associated with ion channel gene mutations.
We are interested in the following sub-topics but not limited to:
• Cardiac electrophysiology using experiment genetic modified animal models.
• Innovation therapy strategies using iPSC-CM models.
Original Research, Systematic Review, Review, Mini Review and Methods articles are welcomed.
The pathophysiological mechanisms of inherited cardiac arrhythmias syndromes have been investigated using experimental model systems, including transgenic murine models, canine heart preparations, zebrafish, and expression systems, to study different gene mutations. The recent development of induced pluripotent stem cell (iPS)-derived cardiomyocytes creates an opportunity to study cardiomyocytes derived from patients and healthy individuals. This Research Topic focuses on basic electrophysiological research using experimental models for unravelling the specific mechanisms, the action of modifier genes, and exploring novel therapeutic strategies.
Genetically modified murine models have allowed for detailed investigation of the in vivo, ex vivo, and in vitro electrophysiological consequences of human mutations associated with inherited cardiac arrhythmias syndromes. However, certain cardiac electrophysiological properties are inherently different between mouse and human. Perfused wedges of canine left or right ventricles rather than a whole heart have been widely used to study cardiac electrophysiology. Zebrafish are relatively easy to study and genetically modify. Ion channel disorders related to repolarization disorders have been successfully modelled in zebrafish. More recently, human-induced pluripotent stem cell-derived (iPSC) cardiomyocytes may recapitulate disease phenotypes in inherited cardiac rhythm disorders. Due to iPSC-CM relative immature phenotypes, caution should be taken when interpreting electrophysiological data from iPSC-derived cardiomyocytes. Nevertheless, it is a promising tool for studying pathophysiology, genotype-phenotype relationship, and pharmacology in cardiac arrhythmia syndromes.
In the last few decades, significant progress has been made in clinical practice and basic research to understand the natural history and mechanisms of the inherited cardiac arrhythmias syndromes. However, those highly potentially life-threatening ventricular arrhythmias and sudden cardiac death, especially in young, still largely remain unexplored. This Research Topic aims to investigate and better understand the mechanisms of causing genes, the action of modifier genes and explore innovation therapy strategies using experiment models. A deeper knowledge of these mechanisms may help in further elucidating the pathophysiology and explore innovation therapy strategies.
This Research Topic focuses on the electrophysiology of the inherited cardiac arrhythmias syndromes, such as LQT, CPVT, Brugada syndrome, SQT, and conduction disorders associated with ion channel gene mutations.
We are interested in the following sub-topics but not limited to:
• Cardiac electrophysiology using experiment genetic modified animal models.
• Innovation therapy strategies using iPSC-CM models.
Original Research, Systematic Review, Review, Mini Review and Methods articles are welcomed.
Keywords: Cardiac channelopathies, inherited cardiac disorders, arrhythmias, Cardiac Electrophysiology, human-induced pluripotent stem cells(hiPSC)
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