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Cold-induced disruption of Na(+) channel slow inactivation underlies paralysis in highly thermosensitive paramyotonia
The Q270K mutation of the skeletal muscle Na(+) channel α subunit (Nav1.4) causes atypical paramyotonia with a striking sensitivity to cold. Attacks of paralysis and a drop in the compound muscle action potential (CMAP) are exclusively observed at cold. To understand the pathogenic process, we studi...
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Main Authors: | , , , , |
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Formato: | Artigo |
Idioma: | Inglês |
Publicado em: |
Blackwell Science Inc
2009
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Assuntos: | |
Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2683958/ https://ncbi.nlm.nih.gov/pubmed/19221125 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1113/jphysiol.2008.165787 |
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