PDZ domain-binding motif regulates cardiomyocyte compartment-specific NaV1.5 channel expression and function.

@article{Shy2014PDZDM,
  title={PDZ domain-binding motif regulates cardiomyocyte compartment-specific NaV1.5 channel expression and function.},
  author={Diana Shy and Ludovic Gillet and Jakob Ogrodnik and Maxime Albesa and Arie O. Verkerk and Rianne Wolswinkel and Jean-S{\'e}bastien Rougier and Julien Barc and Maria C. Essers and Ninda Syam and Roos F J Marsman and Anneke M van Mil and Samuel Rotman and Richard Redon and Connie R. Bezzina and Carol Ann Remme and H. Abriel},
  journal={Circulation},
  year={2014},
  volume={130 2},
  pages={147-60}
}
BACKGROUND Sodium channel NaV1.5 underlies cardiac excitability and conduction. The last 3 residues of NaV1.5 (Ser-Ile-Val) constitute a PDZ domain-binding motif that interacts with PDZ proteins such as syntrophins and SAP97 at different locations within the cardiomyocyte, thus defining distinct pools of NaV1.5 multiprotein complexes. Here, we explored the in vivo and clinical impact of this motif through characterization of mutant mice and genetic screening of patients. METHODS AND RESULTS… CONTINUE READING
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