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Antibodies to potassium channels of PC12 in serum of Isaacs' syndrome: Western blot and immunohistochemical studies.

Abstract
We investigated the pathophysiology of nerve hyperexcitability in a patient with Isaacs' syndrome, who had typical clinical and electromyographic features and responded to plasma exchange. Immunoblotting and immunohistochemistry studies showed that antibodies from this patient reacted with the lysate of a neuronal cell line (PC12). In Western blots, constituents of the patient's serum, particularly immunoglobulin M, reacted with proteins of approximately 50 and 18 kDa, whereas the control serum did not. A cross-linking study with alpha-dendrotoxin (7 kDa) showed a 57 kDa protein-peptide complex. Immunohistochemistry showed that the patient's serum reacted with PC12 cells and human intramuscular nerve axons. Our findings indicate that in Isaac's syndrome nerve hyperexcitability is the result of the immunological involvement of the voltage-dependent potassium channels located along the distal motor nerve or at the nerve terminal.
AuthorsK Arimura, O Watanabe, I Kitajima, M Suehara, S Minato, Y Sonoda, I Higuchi, S Takenaga, I Maruyama, M Osame
JournalMuscle & nerve (Muscle Nerve) Vol. 20 Issue 3 Pg. 299-305 (Mar 1997) ISSN: 0148-639X [Print] United States
PMID9052808 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Antibodies
  • Potassium Channels
Topics
  • Aged
  • Aged, 80 and over
  • Animals
  • Antibodies (analysis)
  • Blotting, Western
  • Fasciculation (immunology, metabolism)
  • Humans
  • Immunohistochemistry (methods)
  • Male
  • Muscles (innervation)
  • Nerve Tissue (metabolism)
  • PC12 Cells (metabolism)
  • Potassium Channels (immunology)
  • Rats
  • Staining and Labeling

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