Pediatr Cardiol. 2019 Oct;40(7):1388-1400. doi: 10.1007/s00246-019-02166-4. Epub 2019 Aug 1.

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

Author information

1
Department of Physiology, University of Kentucky School of Medicine, Lexington, KY, USA.
2
Department of Epidemiology, University of Texas MD Anderson Cancer Center, Houston, TX, USA.
3
Department of Molecular and Functional Genomics, Weis Center for Research, Geisinger Clinic, 100 North Academy Ave 26-18, Danville, PA, 17822, USA.
4
Department of Molecular and Functional Genomics, Weis Center for Research, Geisinger Clinic, 100 North Academy Ave 26-18, Danville, PA, 17822, USA. ammoon@geisinger.edu.
5
Departments of Pediatrics and Human Genetics, University of Utah School of Medicine, Salt Lake City, UT, USA. ammoon@geisinger.edu.

Abstract

In this article, we provide a brief summary of work by us and others to discover the molecular underpinnings of early conduction system development and function. We focus on how the multifunctional protein Tbx3 contributes to acquisition and homeostasis of the tissue-specific properties of the sinoatrial and atrioventricular nodes. We also provide unpublished, preliminary findings supporting the role of Tbx3-regulated alternative RNA processing in the developing conduction system.

KEYWORDS: Channelopathies; Conduction system; Development; Tbx3

PMID: 31372681
 
DOI: 10.1007/s00246-019-02166-4
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