Tbx3-Mediated Regulation of Cardiac Conduction System Development and Function: Potential Contributions of Alternative RNA Processing

The contraction of the heart is orchestrated by the components of the cardiac conduction system (CCS), which initiate and propagate the electrical impulses to coordinately activate the cardiac chambers. In the adult heart, the impulse is generated in the sinoatrial node and activates the atrial myocardium. Slow conduction of the impulse through the atrioventricular node allows for emptying of the atria and filling of the ventricles prior to ventricular contraction. Subsequent fast conduction through the atrioventricular bundle, bundle branches, and Purkinje fibre network activates the ventricular myocardium and causes the ventricles to contract. The development and function of the CCS involves complex regulatory networks of transcription factors acting in stage-, tissue-, and dose-dependent manners. Disrupted function or expression of these factors might lead to impaired development or function of the CCS components, associated with heart failure and sudden death. It is therefore crucial to understand the molecular and cellular mechanisms controlling the complex regulation of CCS development. This chapter summarizes current insight in the development and function of the different compartments of the CCS, and discusses the transcriptional networks underlying these processes.

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Development of the cardiac conduction system

ESC CardioMed ◽

10.1093/med/9780198784906.003.0008 ◽

2018 ◽

pp. 49-52

Author(s):

Jan Hendrik van Weerd ◽

Vincent M. Christoffels

Keyword(s):

Regulatory Networks ◽

Atrioventricular Node ◽

Ventricular Myocardium ◽

Conduction System ◽

Cardiac Conduction ◽

Cardiac Conduction System ◽

Cellular Mechanisms ◽

Fibre Network ◽

Electrical Impulses ◽

And Function

The contraction of the heart is orchestrated by the components of the cardiac conduction system (CCS), which initiate and propagate the electrical impulses to coordinately activate the cardiac chambers. In the adult heart, the impulse is generated in the sinoatrial node and activates the atrial myocardium. Slow conduction of the impulse through the atrioventricular node allows for emptying of the atria and filling of the ventricles prior to ventricular contraction. Subsequent fast conduction through the atrioventricular bundle, bundle branches, and Purkinje fibre network activates the ventricular myocardium and causes the ventricles to contract. The development and function of the CCS involves complex regulatory networks of transcription factors acting in stage-, tissue-, and dose-dependent manners. Disrupted function or expression of these factors might lead to impaired development or function of the CCS components, associated with heart failure and sudden death. It is therefore crucial to understand the molecular and cellular mechanisms controlling the complex regulation of CCS development. This chapter summarizes current insight in the development and function of the different compartments of the CCS, and discusses the transcriptional networks underlying these processes.

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The formation and function of the cardiac conduction system

Development ◽

10.1242/dev.124883 ◽

2016 ◽

Vol 143 (2) ◽

pp. 197-210 ◽

Cited By ~ 85

Author(s):

Jan Hendrik van Weerd ◽

Vincent M. Christoffels

Keyword(s):

Conduction System ◽

Cardiac Conduction ◽

Cardiac Conduction System ◽

And Function

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Development of the Cardiac Conduction System and the Possible Relation to Predilection Sites of Arrhythmogenesis

The Scientific World JOURNAL ◽

10.1100/tsw.2008.40 ◽

2008 ◽

Vol 8 ◽

pp. 239-269 ◽

Cited By ~ 51

Author(s):

M. R. M. Jongbloed ◽

E. A. F. Mahtab ◽

N. A. Blom ◽

M. J. Schalij ◽

A. C. Gittenberger-de Groot

Keyword(s):

Complex System ◽

Current Knowledge ◽

System Development ◽

Expression Patterns ◽

Conduction System ◽

Cardiac Conduction ◽

Cardiac Conduction System ◽

Developing Heart ◽

Complex Process ◽

Embryologic Development

The cardiac conduction system (CCS) encompasses a complex system responsible for the coordinated contraction of the heart. In the developing heart, as well as in the adult heart, tissues of the (putative) CCS are characterized by different properties than the surrounding working myocardium, which can be observed on a histological level, as well as by the expression patterns of several immunohistological and molecular markers. In recent years, many markers have been studied that have helped to elucidate the processes involved in CCS development. It has become clear that multiple genes, cells and their interactions are involved in this complex process. In this article, an overview of the current knowledge of CCS development is supplied. Furthermore, several controversies regarding conduction system development are discussed, as well as the possible significance of embryologic development of the CCS for the development of arrhythmias later in life.

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