Abstract
In health, acetylcholine receptors (AChR) associated with muscle membrane at the endplate are designed to capture acetylcholine molecules released from vesicles at the motor nerve terminals. The AChR, complexed with transmitter, then acts as an ion channel to mediate cationic fluxes into innervated muscles, thereby initiating the chain of events leading to voluntary muscle contraction. Under certain circumstances, however, the AChR may be perturbed in a way that diminishes its ability to translocate ions effectively. Such is the case in myasthenia gravis (MG), an autoimmune disease mediated by antibodies with binding specificity for AChR; upon binding, anti-AChR antibodies may interfere with receptor function to varying degrees, impairing neuromuscular transmission and creating the commonly observed symptoms of weakness and easy fatigability. Much has been learned over the years with regard to the mechanisms underlying MG. However, much is still to be clarified. This is certainly due to the multifactorial nature of the disease: mechanisms for impaired neuromuscular function may be found at several levels. For example, as summarized in Figure 8.1, the induction and severity of disease symptoms might be influenced by the myophysiology of an individual, reflected by minor differences in AChR structure or numbers of receptors found on the post-junctional muscle membrane.
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Krolick, K.A., Thompson, P.A., Zoda, T.E., Mohan, S., Barohn, R.J., Yeh, TM. (1994). Immunological factors that influence disease severity in experimental autoimmune myasthenia gravis. In: Hohlfeld, R. (eds) Immunology of Neuromuscular Disease. Immunology and Medicine Series, vol 24. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-1422-6_8
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