Conti-Fine Bianca M, Milani Monica, Kaminski Henry J
Department of Biochemistry, University of Minnesota, Minneapolis, Minnesota 55455, USA.
J Clin Invest. 2006 Nov;116(11):2843-54. doi: 10.1172/JCI29894.
Myasthenia gravis (MG) is an autoimmune syndrome caused by the failure of neuromuscular transmission, which results from the binding of autoantibodies to proteins involved in signaling at the neuromuscular junction (NMJ). These proteins include the nicotinic AChR or, less frequently, a muscle-specific tyrosine kinase (MuSK) involved in AChR clustering. Much is known about the mechanisms that maintain self tolerance and modulate anti-AChR Ab synthesis, AChR clustering, and AChR function as well as those that cause neuromuscular transmission failure upon Ab binding. This insight has led to the development of improved diagnostic methods and to the design of specific immunosuppressive or immunomodulatory treatments.
重症肌无力(MG)是一种由神经肌肉传递功能障碍引起的自身免疫综合征,其病因是自身抗体与神经肌肉接头(NMJ)处参与信号传导的蛋白质结合。这些蛋白质包括烟碱型乙酰胆碱受体(nicotinic AChR),或较少见的参与乙酰胆碱受体聚集的肌肉特异性酪氨酸激酶(MuSK)。关于维持自身耐受性、调节抗乙酰胆碱受体抗体合成、乙酰胆碱受体聚集及功能的机制,以及抗体结合后导致神经肌肉传递功能障碍的机制,我们已经了解很多。这些认识促使了诊断方法的改进以及特异性免疫抑制或免疫调节治疗方案的设计。