PASSIVELY TRANSFERRED EXPERIMENTAL AUTO-IMMUNE MYASTHENIA-GRAVIS
PASSIVELY TRANSFERRED EXPERIMENTAL AUTO-IMMUNE MYASTHENIA-GRAVIS
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DOI：
10.1212/wnl.29.2.179
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发表时间：
1979-01-01
期刊：
影响因子：
9.9
通讯作者：
LENNON, VA
中科院分区：
文献类型：
--
作者：
ENGEL, AG;SAKAKIBARA, H;LENNON, VA
用免疫球蛋白[Ig]将实验性自身免疫性重症肌无力（EAMG）从慢性EAMG大鼠被动转移到正常受体。IgG和C3[补体成分3]在6 h时定位于终板连接褶皱末端扩张处，富含乙酰胆碱受体（AChR）并被IgG和C3包被的褶皱片段在24 h时脱落到突触间隙，导致突触后膜AChR缺失。许多致敏的突触后区域在第2天被巨噬细胞破坏，但有效的神经-肌肉接触在第5天重建。第10天，终板仍出现结构异常和AChR缺乏，但动物临床痊愈。在第54天，突触后区域的大小仍然减少，突触后AChR略有减少。在整个研究过程中，微型终板电位振幅往往与对AChR反应的突触后膜丰度的形态计量学估计直接变化。补体介导的连接褶皱损伤和突触后区域的调理可以解释这些形态学变化。目前尚不清楚为什么在急性EAMG和慢性EAMG免疫球蛋白诱导的被动转移EAMG中会发生终板的吞噬，而在慢性EAMG中不会发生，在人类疾病中很少发生。
Experimental autoimmune myasthenia gravis (EAMG) was passively transferred with immunoglobulin [Ig] from rats with chronic EAMG to normal recipients. IgG and C3 [complement component 3] were localized on terminal expansions of junctional folds of end-plates by 6 h. Segments of folds rich in acetylcholine receptor (AChR) and coated with IgG and C3 were shed into the synaptic space by 24 h, resulting in AChR deficiency of the postsynaptic membrane. Many sensitized postsynaptic regions were destroyed by macrophages by day 2, but effective nerve-muscle contacts were reestablished by day 5. On day 10, end-plates were still structurally abnormal and showed AChR deficiency, but the animals were clinically recovered. On day 54, postsynaptic regions were still reduced in size, with slight reduction of postsynaptic AChR. Throughout the study, the miniature end-plate potential amplitude tended to vary directly with morphometric estimates of the abundance of the postsynaptic membrane reacting for AChR. Complement-mediated injury to the junctional folds and opsonization of the postsynaptic region can explain the morphologic changes. It is not yet known why phagocytic invasion of the end-plate occurs in acute EAMG and in passively transferred EAMG induced by chronic EAMG immunoglobulin, but not in chronic EAMG and only rarely in the human disease.