Ruff Robert L, Lisak Robert P
Department of Neurology, Case Western University School of Medicine, The Metro Health System, 2500 Metro Health Drive, Cleveland, OH 44109, USA; Department of Neurosciences, Case Western Reserve University, Cleveland, OH, USA.
Department of Neurology, Wayne State University School of Medicine, 8D University Health Center, 4201 St Antoine, Detroit, MI 48201, USA; Department of Biochemistry, Microbiology and Immunology, Wayne State University, Detroit, MI, USA.
Neurol Clin. 2018 May;36(2):275-291. doi: 10.1016/j.ncl.2018.01.001.
This article discusses antibodies associated with immune-mediated myasthenia gravis and the pathologic action of these antibodies at the neuromuscular junctions of skeletal muscle. To explain how these antibodies act, we consider the physiology of neuromuscular transmission with emphasis on 4 features: the structure of the neuromuscular junction; the roles of postsynaptic acetylcholine receptors and voltage-gated Na channels and in converting the chemical signal from the nerve terminal into a propagated action potential on the muscle fiber that triggers muscle contraction; the safety factor for neuromuscular transmission; and how the safety factor is reduced in different forms of autoimmune myasthenia gravis.
本文讨论了与免疫介导的重症肌无力相关的抗体,以及这些抗体在骨骼肌神经肌肉接头处的病理作用。为了解释这些抗体的作用机制,我们着重从四个方面来考虑神经肌肉传递的生理学:神经肌肉接头的结构;突触后乙酰胆碱受体和电压门控钠通道的作用,以及如何将来自神经末梢的化学信号转化为肌肉纤维上的传播动作电位以触发肌肉收缩;神经肌肉传递的安全系数;以及在不同形式的自身免疫性重症肌无力中安全系数是如何降低的。