Nagaishi Akiko, Sakai Waka, Motomura Masakatsu
Department of Neurology, National Hospital Organization Nagasaki-Kawatana Medical Center.
Nihon Rinsho. 2013 May;71(5):876-80.
Patients with myasthenia gravis(MG) are divided into three groups: (1) acetylcholine receptor antibody positive MG: 80%, (2) muscle-specific receptor tyrosine kinase (MuSK) antibody positive MG: 5-10%, and (3) double seronegative MG. In 2011, autoantibodies (Abs) against low-density lipoprotein receptor-related protein 4(Lrp4) were identified in Japanese MG patients and thereafter have been reported in Germany and USA. In other Lrp4 Ab papers, Lrp4 Ab positive sera inhibited agrin-induced aggregation of AChRs in cultured myotubes, suggesting a pathogenic role regarding the dysfunction of the neuromuscular endplate. Anti-MuSK autoantibodies were revealed to block binding of collagen Q (ColQ) to MuSK. Anti-Kv1.4 antibodies targeting alpha-subunits(Kv1.4) of the voltage-gated potassium channel occurs frequently among MG patients with thymoma. Further understandings of neuromuscular junction structure and functions through newly discovered autoantibodies may provide more specific clinical information and treatments in MG.
重症肌无力(MG)患者分为三组:(1)乙酰胆碱受体抗体阳性MG:80%;(2)肌肉特异性受体酪氨酸激酶(MuSK)抗体阳性MG:5 - 10%;(3)双血清阴性MG。2011年,在日本MG患者中发现了针对低密度脂蛋白受体相关蛋白4(Lrp4)的自身抗体(Abs),此后在德国和美国也有相关报道。在其他关于Lrp4抗体的论文中,Lrp4抗体阳性血清可抑制培养肌管中聚集蛋白诱导的乙酰胆碱受体聚集,提示其在神经肌肉终板功能障碍方面的致病作用。抗MuSK自身抗体被发现可阻断胶原蛋白Q(ColQ)与MuSK的结合。靶向电压门控钾通道α亚基(Kv1.4)的抗Kv1.4抗体在伴有胸腺瘤的MG患者中频繁出现。通过新发现的自身抗体进一步了解神经肌肉接头的结构和功能,可能为MG提供更具体的临床信息和治疗方法。