{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T13:05:08Z","timestamp":1782997508733,"version":"3.54.5"},"reference-count":26,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2002,3,28]],"date-time":"2002-03-28T00:00:00Z","timestamp":1017273600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2002,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In the Lambert\u2013Eaton myasthenic syndrome (LEMS), immunoglobulin G (IgG) autoantibodies to presynaptic voltage\u2010gated calcium channels (VGCCs) at the neuromuscular junction lead to a reduction in nerve\u2010evoked release of neurotransmitter and muscle weakness. We have examined the action of LEMS IgGs on cloned human VGCCs stably expressed in transfected human embryonic kidney (HEK293) cell lines: 10\u201313 (\u03b1<jats:sub>1A\u20102<\/jats:sub>, \u03b1<jats:sub>2b<\/jats:sub>\u03b4, \u03b2<jats:sub>4a<\/jats:sub>) and C2D7 (\u03b1<jats:sub>1B\u20101<\/jats:sub> , \u03b1<jats:sub>2b<\/jats:sub>\u03b4, \u03b2<jats:sub>1b<\/jats:sub>). All LEMS IgGs studied showed surface binding to [<jats:sup>125<\/jats:sup>I]\u2010\u03c9\u2010CTx\u2010MVIIC\u2010labeled VGCCs in the \u03b1<jats:sub>1A<\/jats:sub> cell line and two of six IgGs showed surface binding to [<jats:sup>125<\/jats:sup>I]\u2010\u03c9\u2010CTx\u2010GVIA\u2010labeled VGCCs in the \u03b1<jats:sub>1B<\/jats:sub> cell line. We next studied the effect of LEMS IgGs (2 mg\/ml) on whole\u2010cell calcium currents in the \u03b1<jats:sub>1A<\/jats:sub> and \u03b1<jats:sub>1B<\/jats:sub> cell lines. Overnight treatment of \u03b1<jats:sub>1A<\/jats:sub> (10\u201313) cells with LEMS IgGs led to a significant reduction in peak current density without alteration of the current\u2013voltage relationship or the voltage dependence of steady\u2010state inactivation. In contrast, LEMS IgGs did not reduce peak current density in the \u03b1<jats:sub>1B<\/jats:sub> cell line. Overall these data demonstrate the specificity of LEMS IgGs for the \u03b1<jats:sub>1A<\/jats:sub> cell line and suggest that LEMS IgGs bind to and downregulate VGCCs in this cell line. Although several LEMS IgGs can be shown to bind to the \u03b1<jats:sub>1B<\/jats:sub> (C2D7) cell line, no functional effects were seen on this channel. \u00a9 2002 Wiley Periodicals, Inc. 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