Tbx3 is required for normal cardiac conduction system development

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Tbx3 is required for outflow tract development.

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Transcription factor Tbx3 is required for the specification of the atrioventricular conduction system.

The cardiac conduction system consists of distinctive heart muscle cells that initiate and propagate the electric impulse required for coordinated contraction. The conduction system expresses the transcriptional repressor Tbx3, which is required for vertebrate development and controls the formation of the sinus node. In humans, mutations in Tbx3 cause ulnar-mammary syndrome. Here, we investigat...

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Setting the pace: Tbx3 and Tbx18 in cardiac conduction system development.

A vexing problem for clinicians in the management of children and adults with congenital heart disease is the management of the accompanying arrhythmias. In the recent decade, the mutational spectrum associated with human congenital heart disease has begun to come into focus.1 The number of distinct genes implicated in human congenital heart disease is considerable, and a large portion of mutat...

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A large permissive regulatory domain exclusively controls Tbx3 expression in the cardiac conduction system.

RATIONALE The evolutionary conserved Tbx3/Tbx5 gene cluster encodes T-box transcription factors that play crucial roles in the development and homeostasis of the cardiac conduction system in human and mouse. Both genes are expressed in overlapping patterns and function in strictly tissue-specific and dose-dependent manners, yet, their regulation is poorly understood. OBJECTIVE To analyze the ...

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A Molecular Pathway Including Id2, Tbx5, and Nkx2-5 Required for Cardiac Conduction System Development

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ژورنال

عنوان ژورنال: The FASEB Journal

سال: 2008

ISSN: 0892-6638,1530-6860

DOI: 10.1096/fasebj.22.1_supplement.584.6