The T-Box transcription factor Tbx5 is required for the patterning and maturation of the murine cardiac conduction system.

@article{Moskowitz2004TheTT,
  title={The T-Box transcription factor Tbx5 is required for the patterning and maturation of the murine cardiac conduction system.},
  author={Ivan P Moskowitz and Anne Pizard and Vickas V. Patel and Benoit G Bruneau and Jae Bum Kim and Sabina Kupershmidt and Dan M. Roden and Charles I. Berul and Christine E Seidman and Jonathan G Seidman},
  journal={Development},
  year={2004},
  volume={131 16},
  pages={4107-16}
}
We report a critical role for the T-box transcription factor Tbx5 in development and maturation of the cardiac conduction system. We find that Tbx5 is expressed throughout the central conduction system, including the atrioventricular bundle and bundle branch conduction system. Tbx5 haploinsufficiency in mice (Tbx5(del/+)), a model of human Holt-Oram syndrome, caused distinct morphological and functional defects in the atrioventricular and bundle branch conduction systems. In the… CONTINUE READING

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