Lennon V A, Seybold M E, Lindstrom J M, Cochrane C, Ulevitch R
J Exp Med. 1978 Apr 1;147(4):973-83. doi: 10.1084/jem.147.4.973.
An acute phase of experimental autoimmune myasthenia gravis (EAMG) occurs transiently early in the immune response of Lewis rats to nicotinic acetylcholine receptors (AChR) when Bordetella pertussis is used as adjuvant. It is characterized by a destructive cellular attack directed at the postsynaptic membranes of muscle. Acute EAMG can be passively transferred to normal rats by IgG from serum of rats with chronic EAMG. In the present study, acute EAMG, induced either by passive transfer of syngeneic antibodies or by active immmunization, was inhibited in rats depleted of complement by treatment with cobra venom factor (CoF). Furthermore, passive transfer of antibodies in excess of the muscle's content of AChR was without any measurable effect in rats treated with CoF. Although 60% of the muscle's AChR was complexed with antibody, there was no reduction in the muscle's content of AChR, and neuromuscular transmission was not compromised as judged electromyographically by curare sensitivity. These data imply that redistribution, accelerated degradation, and impairment of the ionophore function of AChR, effects of antibodies described in vitro on extrajunctional AChR, do not play a significant role in vivo in impairing neuromuscular transmission in an intact neuromuscular junction. Complement appears to be a critical mediator of anti-AChR antibodies' pathogenicity in vivo.
当使用百日咳博德特氏菌作为佐剂时,实验性自身免疫性重症肌无力(EAMG)的急性期会在Lewis大鼠对烟碱型乙酰胆碱受体(AChR)的免疫反应早期短暂出现。其特征是针对肌肉突触后膜的破坏性细胞攻击。急性EAMG可通过慢性EAMG大鼠血清中的IgG被动转移至正常大鼠。在本研究中,用眼镜蛇毒因子(CoF)处理使补体耗竭的大鼠,由同种抗体被动转移或主动免疫诱导的急性EAMG受到抑制。此外,在经CoF处理的大鼠中,超过肌肉AChR含量的抗体被动转移没有任何可测量的影响。尽管60%的肌肉AChR与抗体形成复合物,但肌肉AChR的含量没有减少,并且通过箭毒敏感性的肌电图判断,神经肌肉传递没有受到损害。这些数据表明,AChR的重新分布、加速降解以及离子载体功能受损,即体外描述的抗体对接头外AChR的作用,在完整神经肌肉接头中损害神经肌肉传递的体内过程中不发挥重要作用。补体似乎是抗AChR抗体在体内致病性的关键介质。