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ANTIBODIES AND RECEPTORS: From Neuromuscular Junction to Central Nervous System.
Neuroscience ( IF 2.9 ) Pub Date : 2020-03-17 , DOI: 10.1016/j.neuroscience.2020.03.009
Angela Vincent 1
Affiliation  

Myasthenia gravis (MG) is a relatively rare neurological disease that is usually associated with antibodies to the acetylcholine receptor (AChR). These antibodies (Abs) cause loss of the AChRs from the neuromuscular junction (NMJ), resulting in muscle weakness that can be life-threatening. Another form of the disease is caused by antibodies to muscle specific kinase (MuSK) that result in impaired AChR clustering and numbers at the NMJ, and may also interfere with presynaptic adaptive mechanisms. Other autoimmune disorders, Lambert Eaton myasthenic syndrome and acquired neuromyotonia, are associated with antibodies to presynaptic voltage-gated calcium and potassium channels respectively. All four conditions can be diagnosed by specific clinical features, electromyography and serum antibody tests, and can be treated effectively by a combination of pharmacological approaches and procedures that reduce the levels of the IgG antibodies. They form the first of a spectrum of diseases in which serum autoantibodies bind to extracellular domains of neuronal proteins throughout the nervous system and lead to constellations of clinical features including paralysis, sensory disturbance and pain, memory loss, seizures, psychiatric disturbance and movement disorders. This review will briefly summarize the ways in which this field has developed, since the 1970s when considerable contributions were made in Ricardo Miledi’s laboratory at UCL.



中文翻译:

抗体和受体:从神经肌肉连接到中枢神经系统。

重症肌无力(MG)是一种相对罕见的神经系统疾病,通常与乙酰胆碱受体(AChR)抗体有关。这些抗体(Abs)导致AChR从神经肌肉接头(NMJ)中丢失,从而导致肌肉无力,这可能危及生命。该疾病的另一种形式是由抗肌肉特异性激酶(MuSK)的抗体引起的,该抗体导致NMJ的AChR簇和数量受损,并且还可能干扰突触前的适应机制。其他自身免疫性疾病(兰伯特·伊顿肌无力综合症和后天性神经性肌强直)分别与突触前电压门控钙和钾通道的抗体有关。可以通过特定的临床特征,肌电图检查和血清抗体检测来诊断所有四种情况,并且可以通过降低IgG抗体水平的药理学方法和程序相结合进行有效治疗。它们构成了一系列疾病中的第一个,其中血清自身抗体与整个神经系统的神经元蛋白的胞外域结合,并导致一系列临床特征,包括麻痹,感觉障碍和疼痛,记忆力减退,癫痫发作,精神病和运动障碍。这篇综述将简要概述自1970年代以来,UCL的里卡多·迈迪(Ricardo Miledi)的实验室做出了巨大贡献以来该领域的发展方式。它们构成了一系列疾病中的第一个,其中血清自身抗体与整个神经系统的神经元蛋白的胞外域结合,并导致一系列临床特征,包括麻痹,感觉障碍和疼痛,记忆力减退,癫痫发作,精神病和运动障碍。这篇综述将简要概述自1970年代以来,UCL的里卡多·迈迪(Ricardo Miledi)的实验室做出了巨大贡献以来该领域的发展方式。它们构成了一系列疾病的第一个疾病,其中血清自身抗体与整个神经系统的神经元蛋白的胞外域结合,并导致一系列临床特征,包括麻痹,感觉障碍和疼痛,记忆力减退,癫痫发作,精神病和运动障碍。这篇综述将简要概述自1970年代以来,UCL的里卡多·迈迪(Ricardo Miledi)的实验室做出了巨大贡献以来该领域的发展方式。

更新日期:2020-03-17
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