Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands; Department of Human Genetics, Leiden University Medical Center, Leiden, the Netherlands.
J Intern Med. 2014 Jan;275(1):12-26. doi: 10.1111/joim.12163. Epub 2013 Nov 29.
Autoantibodies against three different postsynaptic antigens and one presynaptic antigen at the neuromuscular junction are known to cause myasthenic syndromes. The mechanisms by which these antibodies cause muscle weakness vary from antigenic modulation and complement-mediated membrane damage to inhibition of endogenous ligand binding and blocking of essential protein-protein interactions. These mechanisms are related to the autoantibody titre, specific epitopes on the target proteins and IgG autoantibody subclass. We here review the role of specific autoantibody-binding epitopes in myasthenia gravis, their possible relevance to the pathophysiology of the disease and potential implications of epitope mapping knowledge for new therapeutic strategies.
已知针对神经肌肉接头中三个不同的突触后抗原和一个突触前抗原的自身抗体可引起肌无力综合征。这些抗体引起肌肉无力的机制从抗原调节和补体介导的膜损伤到内源性配体结合的抑制和必需蛋白-蛋白相互作用的阻断不等。这些机制与自身抗体滴度、靶蛋白上的特定表位和 IgG 自身抗体亚类有关。我们在此综述了特定自身抗体结合表位在重症肌无力中的作用、它们与疾病病理生理学的可能相关性,以及表位图谱知识对新治疗策略的潜在影响。