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1998 Fiscal Year Final Research Report Summary

The research for voltage-gated K+ channel abnormality in peripheral nerve disorders.

Research Project

Project/Area Number 09670662
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionKagoshima University

Principal Investigator

ARIMURA Kimiyoshi  Faculty of Medicine Kagoshima University, Associate Professor, 医学部, 助教授 (20159510)

Co-Investigator(Kenkyū-buntansha) NAKAGAWA Masanori  University Hospital Kagoshima University, Assistant Professor, 医学部・附属病院, 講師 (50198040)
KAMEYAMA Masaki  Faculty of Medicine Kagoshima University, Professor, 医学部, 教授 (60150059)
Project Period (FY) 1997 – 1998
Keywordsmuscle cramp / myokymia / K+ channel / Na+ channel / Isaacs' syndrome / anti VGKC antibody / western blot / immunoprecipitation
Research Abstract

We studied the suppression of K current by patch clamp method and the existance of anti-VGKC antibodies by western blot (WB) and imrnunoprecipitation (IP) methods in sera from 21 patients with muscle cramp, myokymia and myokymic discharges using cultured cells. In 7 Isaacs' patients, 3 of 7 sera were WB positive and all sera suppressed K current. In 14 other patients, 6 of 14 sera were WB positive and all these sera suppressed K current. Myokymic discharge are frequently seen in patients with the suppression of K current. Only 4 of 14 patients showed IP positive whereas 8 of 14 patients showed WB positive. These findings indicate that anti-VGKC antibodies are not specific for Isaacs' syndrome but are also positive in patients with idiopathic generalized myokymia. IP assay using for detecting anti-channel antibodies in MG and LEMS is not useful for Isaacs' syndrome. From the results of patch clamp studies using of VGKCs (kv 1.1 and 1.6) cDNA transfected cells, the activation kinetics of VGKC were not changed by anti-VGKC antibodies, but total number of VGKC was decreased. Regarding the correlation between anti-ganglioside antibodies (AGA) and conduction block, we could not find the suppression of Na current in sera form 4 GBS patients with positive AGA.We conclude that AGA may not correlate with conduction block due to Na channel suppression.
From our studies, the anti-VGKC antibodies play an important role in neuropathic patients with nerve hyperexcitability and these patients may categorize as potassium channelopathy.

  • Research Products

    (14 results)

All Other

All Publications (14 results)

  • [Publications] 有村公良、長堂竜維、園田至人: "Isaacs症候群及びその近縁疾患で抑制されるK^+ channelのsubtypeについて" 厚生省特定疾患免疫性神経疾患調査研究分科会 平成9年度 研究報告書. 57-59 (1998)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] M.Saito,M.Suehara,A.Nagata,et al.: "Afterdischarges following F waves observed in a patient with tetanus." Electromyogr clin Neurophysiol.38. 377-380 (1998)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] S.Takenaga,K.Arimura,K.Miyamoto,et al.: "Depression of neuromuscular transmission in methymercury-poisoned rats: a glass electode and single fiber electromyography." Acta Neurol Scand. 96. 40-45 (1997)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 有村公良: "筋緊張・けいれんの病態と治療 : Isaacs症候群とstiff-man症候群." 日本内科学会雑誌. 87. 39-44 (1998)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 有村公良: "末梢神経障害と抗K^+チャネル抗体." 最新医学. 53. 171-175 (1998)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 園田至人、有村公良: "Isaacs症候群と抗カリウムチャネル抗体." Clinical Neuroscience. 16. 1178-1181 (1998)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 有村公良、園田至人、渡邊修: "能と神経科学シリーズ9.腫瘍性病変の神経科学" 高倉公明、宮本忠雄 監修, 279-285 (1998)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] K.Arimura, T.Nagado, et al: "Subtype of suppressed K+ channels in Isaacs' syndrome and related diseases." Annual Report of the Neuroimmunological Disorders, Research Committe, The Ministry of Health and Welfare of Japan. 57-59 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] M.Saito, M.Suehara, et al: "Afterdischarges following F waves observed in a patient with tetanus." Electromyogr clin Neurophysiol. 38. 377-380 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] S Takenaga, K.Arimura et al: "Depression of neuromuscular transmission in methylmercury-poisoned rats : a glass electrode and single fiber electromyography." Acta Neurol Scand. 96. 40-45 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] K.Arimura: "The pathophysiology and treatment of muscle stiffness and cramp : Isaacs' syndrome and Stiff-man syndrome." The journal of the Japanese Society of Internal Medicine. 87. 39-44 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] K.Arimura: "Anti-potassium channel antibodies in peripheral nerve." Saisin-Igaku. 53. 171-175 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Y.Sonoda, K.Arimura: "Isaacs' syndrome and anti-potassium channel antibodies." Clinical Neuroscience. 16. 1178-1181 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] K.Arimura, Y.Sonoda, O Watanabe: Isaacs' syndrome. Brain and Neuroscience vol 9, (eds K.Takakura and T.Miyamoto). Medical View Tokyo, 279-285 (1998)

    • Description
      「研究成果報告書概要(欧文)」より

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Published: 1999-12-08  

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