| ○種別 (必須): | □ | 学術論文 (審査論文)
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| ○言語 (必須): | □ | 英語
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| ○組織 (推奨): |
| ○著者 (必須): | 1. | 島谷 佳光
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| 2. | 野寺 裕之
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| 3. | (英) Shibuta Yoshiko (日) (読)
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| 4. | 宮﨑 由道
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| 5. | (英) Misawa Sonoko (日) (読)
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| 6. | (英) Kuwabara Satoshi (日) (読)
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| 7. | 梶 龍兒 ([徳島大学.研究支援・産官学連携センター])
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| ○題名 (必須): | □ | (英) Abnormal gating of axonal slow potassium current in cramp-fasciculation syndrome. (日)
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| ○要約 (任意): | □ | (英) Cramp-fasciculation syndrome (CFS) is a heterogeneous condition with multiple underlying causes. Although dysfunction of slow K(+) channels has been reported in patients with CFS, testing all potential candidates for this problem using conventional in vitro functional analysis would be prohibitively cost- and labor-intensive. However, relatively economical and non-invasive nerve-excitability testing can identify ion channel dysfunction in vivo when combined with numerical modeling. Patients with CFS underwent nerve conduction study, needle electromyography, and nerve excitability testing. Mathematical modeling of axonal properties was applied to identify the pathophysiology. Four patients had distinct electrophysiological findings (i.e., fasciculation potentials, doublet/multiplet motor unit potentials, and sustained F responses); excitability testing showed the following abnormalities: reduction of accommodation during prolonged depolarization, lack of late sub excitability after a supramaximal stimulation, and reduction of the strength-duration time constant. Mathematical modeling showed a loss of voltage-dependence of a slow K(+) current. None of these patients had a mutation in the KCNQ2, 3, or 5 genes. This study showed that patients with CFS might have abnormal kinetics in a slow K(+) current. Nerve-excitability testing may aid the decision to start therapeutic intervention such as administration of slow K(+) channel openers. (日)
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| ○キーワード (推奨): | 1. | (英) Adult (日) (読)
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| 2. | (英) Axons (日) (読)
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| 3. | (英) Electromyography (日) (読)
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| 4. | (英) Female (日) (読)
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| 5. | (英) Humans (日) (読)
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| 6. | (英) Ion Channel Gating (日) (読)
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| 7. | (英) Male (日) (読)
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| 8. | (英) Middle Aged (日) (読)
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| 9. | (英) Neural Conduction (日) (読)
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| 10. | (英) Neuromuscular Diseases (日) (読)
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| 11. | (英) Prospective Studies (日) (読)
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| 12. | (英) Small-Conductance Calcium-Activated Potassium Channels (日) (読)
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| ○発行所 (推奨): |
| ○誌名 (必須): | □ | Clinical Neurophysiology ([International Federation of Clinical Neurophysiology])
(pISSN: 1388-2457, eISSN: 1872-8952)
| ○ISSN (任意): | □ | 1872-8952
ISSN: 1388-2457
(pISSN: 1388-2457, eISSN: 1872-8952) Title: Clinical neurophysiology : official journal of the International Federation of Clinical NeurophysiologyTitle(ISO): Clin NeurophysiolPublisher: Elsevier Ireland Ltd (NLM Catalog)
(Scopus)
(CrossRef)
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| ○巻 (必須): | □ | 126
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| ○号 (必須): | □ | 6
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| ○頁 (必須): | □ | 1246 1254
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| ○都市 (任意): |
| ○年月日 (必須): | □ | 西暦 2014年 9月 28日 (平成 26年 9月 28日)
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| ○URL (任意): |
| ○DOI (任意): | □ | 10.1016/j.clinph.2014.09.013 (→Scopusで検索)
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| ○PMID (任意): | □ | 25304174 (→Scopusで検索)
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| ○備考 (任意): | 1. | (英) PublicationType: Journal Article (日)
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| 2. | (英) PublicationType: Research Support, Non-U.S. Gov't (日)
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