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 language | English |
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Pages (from-to) | 3515-3518 |
Number of pages | 4 |
Journal | NeuroReport |
Volume | 9 |
Issue number | 15 |
Publication status | Published - Oct 26 1998 |
Keywords
- Neurodegeneration
- Oxidative damage
- Patch-clamp
- SH-SY5Y
- Superoxide dismutase
ASJC Scopus subject areas
- Neuroscience(all)