Hohlfeld R, Wekerle H
Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.
Neurol Clin. 1994 May;12(2):331-42.
The experimental work discussed here endorses the hypothesis that in the pathogenesis of MG the initial and essential steps take place within the thymus. Most, if not all, thymuses of MG patients contain B cells capable of producing AChR-specific autoantibody along with appropriate stroma elements. This is especially pertinent in hyperplastic thymuses with germinal centers, which characteristically contain cellular complexes formed by AChR-producing MCs and surrounding interdigitating dendritic cells. The source of the myasthenogenic autoantigen is more complex in thymomas. There are data suggesting that thymoma epithelium express a protein that shares certain peptide epitopes with the AChR alpha chain, although there is no further molecular similarity. A unique type of "molecular self mimicry" could be the starter of thymoma-associated MG.
在重症肌无力(MG)的发病机制中,初始且关键的步骤发生在胸腺内。大多数(即便不是全部)MG患者的胸腺含有能够产生乙酰胆碱受体(AChR)特异性自身抗体的B细胞以及适当的基质成分。这在具有生发中心的增生性胸腺中尤为相关,其特征是含有由产生AChR的肌样细胞(MCs)和周围的交错突细胞形成的细胞复合体。胸腺瘤中致肌无力自身抗原的来源更为复杂。有数据表明胸腺瘤上皮表达一种与AChRα链具有某些肽表位的蛋白质,尽管没有进一步的分子相似性。一种独特类型的“分子自身模拟”可能是胸腺瘤相关MG的起始因素。