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1996 Fiscal Year Final Research Report Summary

The pathomechansim of Isaacs'syndrome.

Research Project

Project/Area Number 07670719
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) KITAJIMA Isao  Faculty of Medicine Kagoshima University Associate Professor, 医学部, 助教授 (50214797)
KAMEYAMA Masaki  Faculty of Medicine Kagoshima University Professor, 医学部, 教授 (60150059)
Project Period (FY) 1995 – 1996
KeywordsIsaacs'syndrome / K+ channel / peripheral nerve hyperexcitability / muscle cramp / myokymia / autoantibody / patch clamp method / immunonohistochemistry
Research Abstract

Purpose :
We studied the pathophysiology of suppression of voltage-dependent potassium channel (VDKC) using patch clamp method in Isaacs'syndrome. We also studied the existence of anti-VDKC autoantibody using molecular biologgical mehods in patients with Isaacs'syndrome and related disordes.
Results :
In additon to PC12, VDCK of NB1 cell co-cultured with patients immunoglobulin and standard medium for 3 days was also suppressed using pathch clamp method. In outward potassium currents, peak current, corresponded to fast K+ channel which is generated immediatery after stimulation, was more suppressed than steady state current, mainly corresponded to slow K+ channel. These findings suggest that antiboy in patients with Isaacs'syndrome seems to mainly block fatst K+ channel. This speculation is concistent with the findins that antigen protein, reacted withpatients'sera, cross-linked with dendrotoxin, which is one of the blocker of fast K+ channel. Furthermore, we investigated the effects of patients'sera on voltage-dependent sodium channel (VDNC) in NB1 cells, however, suppression or activation of VDNC were not seen. Therefore, hyperexcitability of peripheral nerve in Isaacs'syndrome is not due to activation of VDNC but suppresion of VDKC by autoantibody. On the other hand, in some patients with idiopathic generalized myokymia, who shows only myokymia and mild muscle cramp without typical symptome seen in Isaacs'syndrome, antibody which suppressed VDKC of NB1 cell was existed. Furthermore, in early course of Guillain-Barre syndrome, autoantibody to VDKC was also exicted and may correlate to myokymia and dysesthesia seen in early stage of this syndrome.

  • Research Products

    (14 results)

All Other

All Publications (14 results)

  • [Publications] Arimura K,Watanabe O,Kitajima I,Suehara M,et al.: "Antibodies to potassium channels of PC12 in serum of Isaacs'syndrome western blot and immunohistochemical studies." Muscle Nerve. (in press).

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Sonoda Y,Arimura K,Kurono A,Suehara M,et al.: "Serum of Isaacs'syndrome suppresses potassium channels in PC-12 cell lines." Muscle Nerve. 19. 1439-1446 (1996)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Arimura K,Takenaga S,Nakagawa M,Osame M,et al.: "Stimulation single fiber study in a patient with Schwartz-Jampel syndrome." J Neurol Neurosurg Psychiatry. 61. 425-426 (1996)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 有村公良: "末梢神経の興奮性異常:myokymia,neuromyotoniaの病態機序" 脳波と筋電図. 24. 284-292 (1996)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 有村公良: "Myokymiaとその周辺(I)" 臨床脳波. 38. 862-866 (1996)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] 有村公良: "Myokymiaとその周辺(II)" 臨床脳波. 39. 50-54 (1996)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Arimura K,Watanabe O,Sonoda Y,Suehara M,et al.: "Autoimmune mechanism in Isaacs'syndrome.An approach to diseases.-Immunology,Hematology,Cancer-." Kyushu University Press, 171 (1996)

    • Description
      「研究成果報告書概要(和文)」より
  • [Publications] Arimura K,Watanabe O,Kitajima I,et al.: "Antibodies to potassium channels of PC12 in serum of Isaacs'syndrome : western blot and immunohistochemical studies." Muscle Nerve. (in press.).

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Sonoda Y,Arimura K,Kurono A,et al.: "Serum of Isaacs'syndrome suppresses potassium channels in PC-12 cell lines." Muscle Nerve. 19. 1439-1446 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Arimura K,Takenaga S,Nakagawa M,et al.: "Stimulation single fiber study in a patient with Schwartz-Jampel syndrome." J Neurol Neurosurg Psychiatry. 61. 425-426 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Arimura K.: "Hyperexcitability of peripheral motor nerve : The pathophysiology of myokymia and neuromyotonia." Jpn J EEG EMG. 24. 284-292 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Arimura K.: "Myokymia and other positive phenomena of peripheral nerve origin (I)." Clinical encephalography (Japan). 38. 862-866 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Arimura K.: "Myokymia and other positive phenomena of peripheral nerve origin (II)." Clinical encephalography (Japan). 39. 50-54 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
  • [Publications] Arimura K.: Autoimmune mechanism in Isaacs'syndrome. An approach to diseases. -Immunology, Hematology, Cancer-. Kyushu University Press, 171 (1996)

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

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Published: 1999-03-09  

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