Literature DB >> 8874402

Serum of Isaacs' syndrome suppresses potassium channels in PC-12 cell lines.

Y Sonoda1, K Arimura, A Kurono, M Suehara, M Kameyama, S Minato, A Hayashi, M Osame.   

Abstract

Blockage of K+ channels in nerve terminals by immunoglobulin is the speculated pathomechanism of Isaacs' syndrome. Using patch-clamp technique (whole-cell clamp), we investigated the effects on K+ current of serum taken from 2 patients with Isaacs' syndrome employing the clonal cell line PC-12. The addition of a patient's serum to the perfusion solution had little effect on the K+ current of P-12 cells. In contrast, K+ current was reduced by 25-80% when cells were cultured for 3-6 days with 2% serum as compared to control serum values. Suppression of the K+ current appears to develop gradually over the period of culture. Our results suggest that the pathomechanism of Isaacs' syndrome is caused by K+ channel suppression via a humoral factor(s) in the serum, which subsequently induces nerve hyperexcitability.

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Year:  1996        PMID: 8874402     DOI: 10.1002/mus.880191102

Source DB:  PubMed          Journal:  Muscle Nerve        ISSN: 0148-639X            Impact factor:   3.217


  5 in total

1.  Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channel.

Authors:  K Dedek; B Kunath; C Kananura; U Reuner; T J Jentsch; O K Steinlein
Journal:  Proc Natl Acad Sci U S A       Date:  2001-09-25       Impact factor: 11.205

2.  A reverse genetics and genomics approach to gene paralog function and disease: Myokymia and the juxtaparanode.

Authors:  Dana Marafi; Nina Kozar; Ruizhi Duan; Stephen Bradley; Kenji Yokochi; Fuad Al Mutairi; Nebal Waill Saadi; Sandra Whalen; Theresa Brunet; Urania Kotzaeridou; Daniela Choukair; Boris Keren; Caroline Nava; Mitsuhiro Kato; Hiroshi Arai; Tawfiq Froukh; Eissa Ali Faqeih; Ali M AlAsmari; Mohammed M Saleh; Filippo Pinto E Vairo; Pavel N Pichurin; Eric W Klee; Christopher T Schmitz; Christopher M Grochowski; Tadahiro Mitani; Isabella Herman; Daniel G Calame; Jawid M Fatih; Haowei Du; Zeynep Coban-Akdemir; Davut Pehlivan; Shalini N Jhangiani; Richard A Gibbs; Satoko Miyatake; Naomichi Matsumoto; Laura J Wagstaff; Jennifer E Posey; James R Lupski; Dies Meijer; Matias Wagner
Journal:  Am J Hum Genet       Date:  2022-08-09       Impact factor: 11.043

3.  Autoimmune channelopathies of the nervous system.

Authors:  Kleopas A Kleopa
Journal:  Curr Neuropharmacol       Date:  2011-09       Impact factor: 7.363

Review 4.  Autoimmune synaptopathies.

Authors:  Sarah J Crisp; Dimitri M Kullmann; Angela Vincent
Journal:  Nat Rev Neurosci       Date:  2016-02       Impact factor: 34.870

Review 5.  Neuro-glial interactions at the nodes of Ranvier: implication in health and diseases.

Authors:  Catherine Faivre-Sarrailh; Jérôme J Devaux
Journal:  Front Cell Neurosci       Date:  2013-10-29       Impact factor: 5.505

  5 in total

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