Communications Biology (May 2022)

Functional correlates of clinical phenotype and severity in recurrent SCN2A variants

  • Géza Berecki,
  • Katherine B. Howell,
  • Jacqueline Heighway,
  • Nelson Olivier,
  • Jill Rodda,
  • Isabella Overmars,
  • Danique R. M. Vlaskamp,
  • Tyson L. Ware,
  • Simone Ardern-Holmes,
  • Gaetan Lesca,
  • Michael Alber,
  • Pierangelo Veggiotti,
  • Ingrid E. Scheffer,
  • Samuel F. Berkovic,
  • Markus Wolff,
  • Steven Petrou

DOI
https://doi.org/10.1038/s42003-022-03454-1
Journal volume & issue
Vol. 5, no. 1
pp. 1 – 13

Abstract

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A comprehensive biophysical analysis of disease-associated mutations in the voltage-gated sodium channel gene, SCN2A, suggests that dynamic action potential clamp may be a better predictor than voltage clamp of how these mutations alter neuronal excitability, though other approaches are needed to predict severity.