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肌萎缩侧索硬化(ALS)至今病因未明。本文综述关于抗钙离子通道抗体与ALS机制的研究。ALS IgG可与L-VGCC的膜外部分特异性结合起反应,使L-VGCC的钙离子峰流量减少,电荷活动减少。ALS患者中大多含有抗L-VGCC抗体,抗体效价与ALS病情进展速度呈正相关。ALSIgG与L-VGCC的α_1亚单位特异性结合。活检发现ALS患者骨骼肌中的L-VGCC结合动力学有特征性改变。综上所述,抗L-VGCC抗体与ALS发病机制有关。但这是原发的还是继发的,如果是原发的又是怎样起始运动神经元的病变?都有待进一步研究。
Amyotrophic lateral sclerosis (ALS) so far the etiology. This review summarizes the mechanisms of anti-calcium channel antibodies and ALS. ALS IgG can react specifically with the extracellular portion of L-VGCC to reduce the peak of calcium ion in L-VGCC and reduce the charge activity. Most ALS patients contain anti-L-VGCC antibody, antibody titer and ALS progression rate was positively correlated. ALSIgG specifically binds to the α_1 subunit of L-VGCC. Biopsies revealed a characteristic change in L-VGCC binding kinetics in ALS patients. In summary, anti-L-VGCC antibody and ALS pathogenesis. But this is the primary or secondary, if it is the primary motor neuron is how the disease? Need to be further studied.