Nav1.1 mutations cause febrile seizures associated with afebrile partial seizures

Neurology. 2001 Aug 28;57(4):703-5. doi: 10.1212/wnl.57.4.703.

Abstract

Recent evidence has suggested that the neuronal voltage-gated sodium channel alpha(1)-subunit gene (Na(v)1.1: SCN1A) is responsible for generalized epilepsy with febrile seizures plus (GEFS+2). Here the authors report two novel disease mutations of Na(v)1.1 in patients with febrile seizures associated with afebrile partial seizures. One is a Val1428Ala substitution in the pore-forming region, and the other is Ala1685Val in the transmembrane helix. These results support the previous findings and contribute to the reliable diagnosis of epilepsy.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Child
  • Epilepsies, Partial / genetics*
  • Female
  • Humans
  • Male
  • Molecular Sequence Data
  • Mutation, Missense / genetics*
  • NAV1.1 Voltage-Gated Sodium Channel
  • Nerve Tissue Proteins / genetics*
  • Pedigree
  • Seizures, Febrile / genetics*
  • Sodium Channels / genetics*

Substances

  • NAV1.1 Voltage-Gated Sodium Channel
  • Nerve Tissue Proteins
  • SCN1A protein, human
  • Sodium Channels