Myasthenia gravis: past, present, and future
Bianca M. Conti-Fine, … , Monica Milani, Henry J. Kaminski
Bianca M. Conti-Fine, … , Monica Milani, Henry J. Kaminski
Published November 1, 2006
Citation Information: J Clin Invest. 2006;116(11):2843-2854. https://doi.org/10.1172/JCI29894.
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Science in Medicine

Myasthenia gravis: past, present, and future

Abstract

Myasthenia gravis (MG) is an autoimmune syndrome caused by the failure of neuromuscular transmission, which results from the binding of autoantibodies to proteins involved in signaling at the neuromuscular junction (NMJ). These proteins include the nicotinic AChR or, less frequently, a muscle-specific tyrosine kinase (MuSK) involved in AChR clustering. Much is known about the mechanisms that maintain self tolerance and modulate anti-AChR Ab synthesis, AChR clustering, and AChR function as well as those that cause neuromuscular transmission failure upon Ab binding. This insight has led to the development of improved diagnostic methods and to the design of specific immunosuppressive or immunomodulatory treatments.

Authors

Bianca M. Conti-Fine, Monica Milani, Henry J. Kaminski

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Figure 3

Cytokine network and cells involved in the pathogenesis and immunoregulation of MG.

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Cytokine network and cells involved in the pathogenesis and immunoregula...
Th1 cytokines stimulate production of IgG subclasses that bind and activate complement effectively, whereas Th2 cytokines stimulate the production of Ig classes and IgG subclasses that do not. The Th2 cytokine IL-4 is also a differentiation factor for Th3 cells, immunosuppressive cells that secrete TGF-β. The Th1 cytokine IFN-γ stimulates expression of MHC class II molecules on the muscle cell membrane, thus facilitating presentation of muscle AChR. The IL-18 secreted by APCs favors the differentiation of Th1 cells both directly and indirectly through the action of NK cells. CD1-d–restricted NKT cells can activate Tregs, thereby inhibiting autoimmune processes. See text for further details.