Kyriatsoulis A, Manns M, Gerken G, Lohse A W, Maelicke A, Wessler I, Reske K, Meyer zum Büschenfelde K H
I. Medizinische Klinik und Poliklinik, Johannes Gutenberg-Universität Mainz, F.R.G.
J Hepatol. 1988 Jun;6(3):283-90. doi: 10.1016/s0168-8278(88)80044-8.
Although the presence of anti-mitochondrial antibodies is the main characteristic of primary biliary cirrhosis (PBC), other autoantibodies have been described in this disease. This study employs immunoblot methods to test whether the sera of PBC patients also contain antibodies directed against nicotinic acetylcholine receptors (AChR). We show that the majority of patients' sera indeed react with AChR just as sera of myasthenic patients do. In contrast, however, these anti-AChR antibodies do not lead to significant clinical symptoms of myasthenia. In all cases studied, PBC sera recognized a protein with the molecular weight of the alpha-chain of acetylcholine receptor (40 kDa). In addition, with both liver mitochondria and AChR-rich membranes as antigens, PBC sera reacted with proteins with apparent molecular weights around 68 kDa and the same pI values. This protein is not present in purified AChR preparations. These data suggest structural, if not functional, relationships between membrane components of liver mitochondria and muscle endplates.
尽管抗线粒体抗体的存在是原发性胆汁性肝硬化（PBC）的主要特征，但该疾病中也发现了其他自身抗体。本研究采用免疫印迹法检测PBC患者血清中是否也含有抗烟碱型乙酰胆碱受体（AChR）抗体。我们发现，大多数患者血清确实与AChR发生反应，就像重症肌无力患者的血清一样。然而，与之相反的是，这些抗AChR抗体并未导致明显的重症肌无力临床症状。在所有研究病例中，PBC血清识别出一种分子量与乙酰胆碱受体α链（40 kDa）相同的蛋白质。此外，以肝线粒体和富含AChR的膜作为抗原时，PBC血清与表观分子量约为68 kDa且等电点相同的蛋白质发生反应。这种蛋白质不存在于纯化的AChR制剂中。这些数据表明，肝线粒体和肌肉终板的膜成分之间即使不存在功能关系，也存在结构关系。
