Voltage-activated sodium currents in a cell line expressing a Cu,Zn superoxide dismutase typical of familial ALS

Cristina Zona, Alberto Ferri, Roberta Gabbianelli, Nicola B. Mercuri, Giorgio Bernardi, Giuseppe Rotilio, Maria Teresa Carri

Research output: Contribution to journalArticle

Abstract

The whole-cell configuration of the patch-clamp recording was used to study the voltage-dependent Na+ currents in a model system for the familial form of amyotrophic lateral sclerosis (ALS) associated with mutations in Cu,Zn superoxide dismutase. Here we report that the amplitude of voltage- gated Na+ currents is significantly reduced in cell lines expressing mutant Cu,Zn superoxide dismutase G93A when compared with the parental, untransfected cell line and to a cell line expressing the wild-type enzyme. This effect is associated with a shift toward positive values of the steady- state inactivation curve of the Na+ currents. These results indicate that expression of a Cu,Zn superoxide dismutase typical of patients affect with familial ALS influence the functionality of the voltage-dependent Na+ channels; this effect may contribute to the pathogenesis of the disease.

Original languageEnglish
Pages (from-to)3515-3518
Number of pages4
JournalNeuroReport
Volume9
Issue number15
Publication statusPublished - Oct 26 1998

Keywords

  • Neurodegeneration
  • Oxidative damage
  • Patch-clamp
  • SH-SY5Y
  • Superoxide dismutase

ASJC Scopus subject areas

  • Neuroscience(all)

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