期刊
JOURNAL OF THE NEUROLOGICAL SCIENCES
卷 333, 期 1-2, 页码 60-67出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.jns.2012.12.025
关键词
Myasthenia gravis; Thymus; Acetylcholine receptor; Inflammation; Experimental model
资金
- NIH [AI-50058]
Myasthenia gravis is (MG) a prototypic autoimmune disease; the immune effector mechanisms and autoantigenic target have been delineated. However, the events that lead to the abrogation of self-tolerance to neuromuscular acetylcholine receptors (nAChRs) remain a mystery. The thymus gland has long been considered to hold the key to solving this mystery, although the nature of its involvement remains to be elucidated. The nAChR was one of the first self-proteins associated with a defined autoimmune disease that was found to be expressed on thymic stromal populations. The studies described herein represent our efforts to determine how this promiscuous autoantigen expression may be involved in the immunopathogenesis of MG. We review our work, characterizing the expression of the nAChR alpha subunit in the thymus, and advance a hypothesis and experimental model, which explore how intrathymic expression of this autoantigen may contribute to the immunopathogenesis of this autoimmune disease. (C) 2013 Elsevier B.V. All rights reserved.
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