Acetylcholine Receptor-Expressing Fibroblasts

  • Toni Claudio
Conference paper
Part of the NATO ASI Series book series (volume 38)

Abstract

Two key and as yet unresolved questions concerning the autoimmune disease myasthenia gravis (MG) are: 1) what initiates the autoimmune response, and 2) what sustains it. It is known that autoantibodies to the acetylcholine receptor (AChR) can be detected in MG patient sera, that the disease symptoms can be passively transferred from humans to mice with the IgG fraction from MG patient sera (Drachman et al. 1976), that the AChR can induce experimental autoimmune myasthenia gravis (EAMG) in test animals (reviewed in Lindstrom 1985; Newsom-Davis 1986; Engel 1987), and that the AChR is the only protein at endplates which is capable of inducing EAMG (Claudio & Raftery 1980). The importance of the AChR in this disease cannot be disputed, but what role does it play in the initiation and/or maintenance of the disease. Are there specific epitopes on the AChR that are required for induction? It has been suggested that alterations of AChRs on myoid cells in the thymus might serve to break tolerance and initiate the autoimmune response directed against AChRs. Penicillamine has been shown to react with AChRs and to enhance EAMG (Bever et al. 1984). If alterations in the AChR can cause MG, do they occur by an exogenous or an endogenous mechanism? An endogenous mechanism acting directly on AChRs could have several sites of action. Various posttranslational modifications of the AChR are known to occur naturally, including disulfide-bond formation, glycosylation, phosphorylation, and fatty acylation.

Keywords

Leukemia Sedimentation Choline Sine Acetylcholine 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1990

Authors and Affiliations

  • Toni Claudio
    • 1
  1. 1.Department of Cellular & Molecular PhysiologyYale University School of MedicineNew HavenUSA

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