Appel S H, Elias S B, Chauvin P
Fed Proc. 1979 Sep;38(10):2381-5.
Myasthenia gravis is an autoimmune disease of man characterized by remitting and relapsing muscle fatigability. Although the etiology and pathogenesis are incompletely understood, the presence of circulating antibodies directed against the nicotinic acetylcholine (ACh) receptor in 80--90% of patients with myasthenia gravis and the identification of immune complexes at their neuromuscular junction have helped explain the altered neuromuscular transmission. The ACh receptor antibodies do not block access of ACh to the receptor, but do decrease the number of receptors by accelerating their degradation both in rat myotube cultures and in vivo models. In vitro these antibodies play a major role in myasthenia gravis. However, correlations of antibody titers with the clinical state following thymectomy or in neonatal myasthenia suggest that host factors may be equally important in determining whether the ACh receptor antibodies will result in clinical myasthenia.
重症肌无力是一种人类自身免疫性疾病,其特征为肌肉疲劳呈缓解与复发交替状态。尽管病因和发病机制尚未完全明了,但80%至90%的重症肌无力患者体内存在针对烟碱型乙酰胆碱(ACh)受体的循环抗体,并且在其神经肌肉接头处发现了免疫复合物,这有助于解释神经肌肉传递的改变。ACh受体抗体并不阻碍ACh与受体的结合,但在大鼠肌管培养物和体内模型中,通过加速受体降解来减少受体数量。在体外,这些抗体在重症肌无力中起主要作用。然而,胸腺切除术后或新生儿重症肌无力中抗体滴度与临床状态的相关性表明,宿主因素在决定ACh受体抗体是否会导致临床重症肌无力方面可能同样重要。