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

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

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 We also provide unpublished, preliminary findings supporting the role of Tbx3-regulated alternative RNA processing in the developing conduction system.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.

Publication types

  • Review

MeSH terms

  • Animals
  • Atrioventricular Node / physiopathology
  • Gene Expression
  • Gene Expression Regulation, Developmental*
  • Heart Conduction System / embryology
  • Heart Conduction System / metabolism*
  • Humans
  • RNA / metabolism
  • T-Box Domain Proteins

Substances

  • T-Box Domain Proteins
  • TBX3 protein, human
  • Tbx3 protein, mouse
  • RNA