rdf:type |
|
lifeskim:mentions |
|
pubmed:issue |
15
|
pubmed:dateCreated |
1999-2-25
|
pubmed:abstractText |
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.
|
pubmed:language |
eng
|
pubmed:journal |
|
pubmed:citationSubset |
IM
|
pubmed:chemical |
|
pubmed:status |
MEDLINE
|
pubmed:month |
Oct
|
pubmed:issn |
0959-4965
|
pubmed:author |
|
pubmed:issnType |
Print
|
pubmed:day |
26
|
pubmed:volume |
9
|
pubmed:owner |
NLM
|
pubmed:authorsComplete |
Y
|
pubmed:pagination |
3515-8
|
pubmed:dateRevised |
2006-11-15
|
pubmed:meshHeading |
pubmed-meshheading:9855309-4-Aminopyridine,
pubmed-meshheading:9855309-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:9855309-Cadmium,
pubmed-meshheading:9855309-Calcium,
pubmed-meshheading:9855309-Electric Stimulation,
pubmed-meshheading:9855309-Humans,
pubmed-meshheading:9855309-Ion Channel Gating,
pubmed-meshheading:9855309-Mutation,
pubmed-meshheading:9855309-Nerve Degeneration,
pubmed-meshheading:9855309-Neuroblastoma,
pubmed-meshheading:9855309-Neurons,
pubmed-meshheading:9855309-Oxidative Stress,
pubmed-meshheading:9855309-Patch-Clamp Techniques,
pubmed-meshheading:9855309-Potassium,
pubmed-meshheading:9855309-Sodium,
pubmed-meshheading:9855309-Sodium Channels,
pubmed-meshheading:9855309-Superoxide Dismutase,
pubmed-meshheading:9855309-Tumor Cells, Cultured
|
pubmed:year |
1998
|
pubmed:articleTitle |
Voltage-activated sodium currents in a cell line expressing a Cu,Zn superoxide dismutase typical of familial ALS.
|
pubmed:affiliation |
Dipartimento di Neuroscienze, Università di Roma Tor Vergata, Rome, Italy.
|
pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
|