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Antibodies to potassium channels pc12 in serum of Isaacs' syndrome: Western blot and immunohistochemical studies
Author(s) -
Arimura Kimiyoshi,
Watanabe Osamu,
Kitajima Isao,
Suehara Masahito,
Minato Seiichi,
Sonoda Yoshito,
Higuchi Itsuro,
Takenaga Satoshi,
Maruyama Ikuro,
Osame Mitsuhiro
Publication year - 1997
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/(sici)1097-4598(199703)20:3<299::aid-mus6>3.0.co;2-6
Subject(s) - immunohistochemistry , western blot , antibody , motor nerve , blot , potassium channel , medicine , pathology , pathophysiology , immunology , microbiology and biotechnology , chemistry , endocrinology , biology , anatomy , biochemistry , gene
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 at 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. © 1997 John Wiley & Sons, Inc. Muscle Nerve , 20, 299–305, 1997.