Bender A N, Ringel S P, Engel W K, Vogel Z, Daniels M P
Ann N Y Acad Sci. 1976;274:20-30. doi: 10.1111/j.1749-6632.1976.tb47673.x.
The IPBT method has made it possible to precisely visualize the AChR. Normal distribution of AChR is at the peaks of the postjunctional folds of the muscle sarcolemmal membrane with a small amount present on the axonal tip as well. Denervated muscle fibers have extrajunctional AChR. In MG, there are also denervated-appearing fibers but these do not have extrajuctional AChR with the IPBT stain. To explain this, we have been able to demonstrate a serum factor capable of blocking the binding of alpha-BuTx to the AChR and have shown for the first time that this factor is capable of acting at the neuromuscular junction itself. This blocking factor may play a major role in causing the weakness of MG.
免疫过氧化物酶-抗生物素蛋白-生物素技术（IPBT）使得精确观察乙酰胆碱受体（AChR）成为可能。AChR的正常分布位于肌细胞膜突触后皱襞的顶端，在轴突末梢也有少量存在。失神经支配的肌纤维有突触外AChR。在重症肌无力（MG）中，也有看似失神经支配的纤维，但用IPBT染色时这些纤维没有突触外AChR。为了解释这一点，我们已经能够证明一种血清因子能够阻断α-银环蛇毒素（alpha-BuTx）与AChR的结合，并且首次表明这种因子能够在神经肌肉接头本身起作用。这种阻断因子可能在导致MG肌无力方面起主要作用。
