Vincent A
Department of Clinical Neurology, John Radcliffe Hospital, Oxford, United Kingdom.
Brain Behav Immun. 1988 Dec;2(4):346-51. doi: 10.1016/0889-1591(88)90039-6.
Myasthenia gravis (MG) is an autoimmune disease in which the immune system attacks the nicotinic acetylcholine receptors (AChRs) of the neuromuscular junction. Anti-AChR antibodies are present in 85% of patients and bind to distinct epitopes on the surface of the AChR alpha subunits, as defined by competition with monoclonal anti-AChR antibodies. There are at least three types of the disease, defined by thymic histology, age of onset, and HLA associations, and anti-AChR antibodies show some differences in fine specificity between those with thymic hyperplasia and those with thymic tumors. Peripheral blood lymphocytes from MG patients contain T lymphocytes specifically sensitized to AChR. These are stimulated by purified Torpedo AChR and some human alpha subunit synthetic peptides. The T and B cell epitopes on the primary sequence of the alpha subunit are currently being mapped using recombinant human AChR subunit fragments.
重症肌无力(MG)是一种自身免疫性疾病,免疫系统会攻击神经肌肉接头处的烟碱型乙酰胆碱受体(AChRs)。85%的患者体内存在抗AChR抗体,这些抗体与AChRα亚基表面不同的表位结合,这是通过与单克隆抗AChR抗体竞争来定义的。该疾病至少有三种类型,根据胸腺组织学、发病年龄和HLA关联来定义,抗AChR抗体在胸腺增生患者和胸腺肿瘤患者之间的精细特异性上存在一些差异。MG患者的外周血淋巴细胞含有对AChR特异性致敏的T淋巴细胞。这些细胞受到纯化的电鳐AChR和一些人α亚基合成肽的刺激。目前正在使用重组人AChR亚基片段绘制α亚基一级序列上的T细胞和B细胞表位。