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.

Zona, C., Ferri, A., Gabbianelli, R., Mercuri, N.b., Bernardi, G., Rotilio, G., et al. (1998). Voltage-activated sodium currents in a cell line expressing a Cu,Zn superoxide dismutase typical of familial ALS. NEUROREPORT, 9(15), 3515-3518 [10.1097/00001756-199810260-00033].

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

ZONA, CRISTINA;MERCURI, NICOLA BIAGIO;BERNARDI, GIORGIO;ROTILIO, GIUSEPPE;CARRI', MARIA TERESA
1998-10-26

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.
26-ott-1998
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore BIO/10 - BIOCHIMICA
Settore BIO/09 - FISIOLOGIA
English
Con Impact Factor ISI
Superoxide Dismutase; Ion Channel Gating; Calcium; Humans; Potassium; Electric Stimulation; Neuroblastoma; Sodium Channels; Sodium; Nerve Degeneration; Patch-Clamp Techniques; Tumor Cells, Cultured; Amyotrophic Lateral Sclerosis; Neurons; Oxidative Stress; 4-Aminopyridine; Cadmium; Mutation
Zona, C., Ferri, A., Gabbianelli, R., Mercuri, N.b., Bernardi, G., Rotilio, G., et al. (1998). Voltage-activated sodium currents in a cell line expressing a Cu,Zn superoxide dismutase typical of familial ALS. NEUROREPORT, 9(15), 3515-3518 [10.1097/00001756-199810260-00033].
Zona, C; Ferri, A; Gabbianelli, R; Mercuri, Nb; Bernardi, G; Rotilio, G; Carri', Mt
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/52699
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