Delbono O, García J, Appel S H, Stefani E
Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, Texas 77030.
Am J Physiol. 1991 Jun;260(6 Pt 1):C1347-51. doi: 10.1152/ajpcell.1991.260.6.C1347.
Amyotrophic lateral sclerosis (ALS) is a devastating human disease of upper and lower motoneurons. We studied the action of the immunoglobulin G (IgG) from ALS and disease control patients on dihydropyridine (DHP)-sensitive Ca2+ channels in single mammalian skeletal muscle fibers with the double Vaseline gap technique. The peak of the Ca2+ current (ICa) and the charge movement were reduced when the fibers were incubated in ALS IgG. These effects were lost when the IgG was boiled or adsorbed with skeletal tubular membranes. ALS IgG reduced skeletal muscle ICa in a similar fashion as nifedipine; the ICa blockade was voltage dependent, and the associated charge movement was reduced. These observations suggest that IgG from ALS patients reacts with the skeletal muscle DHP-sensitive Ca2+ channels or some associated regulatory moiety.
肌萎缩侧索硬化症（ALS）是一种影响上下运动神经元的毁灭性人类疾病。我们使用双凡士林间隙技术，研究了来自ALS患者和疾病对照患者的免疫球蛋白G（IgG）对单个哺乳动物骨骼肌纤维中二氢吡啶（DHP）敏感的Ca2+通道的作用。当纤维在ALS IgG中孵育时，Ca2+电流（ICa）峰值和电荷移动减少。当IgG煮沸或用骨骼肌管状膜吸附时，这些效应消失。ALS IgG以与硝苯地平类似的方式降低骨骼肌ICa；ICa阻断是电压依赖性的，且相关的电荷移动减少。这些观察结果表明，ALS患者的IgG与骨骼肌DHP敏感的Ca2+通道或某些相关调节部分发生反应。
