Acetylcholine receptor-alpha subunit expression in myasthenia gravis: a role for the autoantigen in pathogenesis?
Acetylcholine receptor-alpha subunit expression in myasthenia gravis: a role for the autoantigen in pathogenesis?
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DOI：
10.1002/mus.21371
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发表时间：
2009-08
期刊：
影响因子：
3.4
通讯作者：
Meriggioli, Matthew N.
中科院分区：
文献类型：
--
作者：
Sheng, Jian Rong;Li, Liang Cheng;Prabhakar, Bellur S.;Meriggioli, Matthew N.
关键词：
以往的研究表明，重症肌无力（MG）和实验性MG（EAMG）中AChR α（AChR-α）亚单位转录本的表达增加，但尚未研究这种过表达的功能特性。我们检测了不同疾病严重程度的EAMG小鼠肌肉组织中AChR-α的mRNA和蛋白表达，以及α-银环蛇毒素标记模式。AChR-α在重度EAMG小鼠终板中的表达显著增加，但其表达明显超过α-银环蛇毒素标记。这种“异常表达”发生在形态学终板损伤的小鼠中，这些小鼠肌肉中补体和免疫球蛋白沉积的模式似乎反映了AChR-α表达的模式。重症肌无力患者AChR功能丧失后，AChR-α mRNA转录增加，但表达的蛋白质是“功能惰性的”，不能补偿AChR的丧失。这种增强的AChR表达可能在驱动持续的自身免疫反应中发挥作用。
Previous studies have shown increased expression of AChR alpha (AChR-α) subunit transcripts in myasthenia gravis (MG) and experimental MG (EAMG) but have not examined the functional properties of this overexpression. We examined the mRNA and protein expression of AChR-α, as well as the pattern of α-bungarotoxin labeling in muscle tissue from EAMG mice with varying disease severity. AChR-α expression was considerably increased in endplates from mice with severe EAMG, but it was distinct and greatly in excess of α-bungarotoxin labeling. This “aberrant expression” occurred in mice with morphologic endplate damage, and the pattern of complement and immunoglobulin deposition in muscle from these mice appeared to mirror the pattern of AChR-α expression. The loss of functional AChR in severe MG increases transcription of AChR-α mRNA, but the expressed protein is “functionally inert,” failing to compensate for loss of AChR. This enhanced expression of AChR may play a role in driving the ongoing autoimmune response.
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