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Unravelling the pathogenesis of myasthenia gravis

Abstract

Myasthenia gravis is a relatively rare neurological disease that is associated with loss of the acetylcholine receptors that initiate muscle contraction. This results in muscle weakness, which can be life-threatening. The story of how both the physiological basis of the disease and the role of acetylcholine-receptor-specific antibodies were determined is a classic example of the application of basic science to clinical medicine, and it has provided a model for defining other antibody-mediated disorders of the peripheral and central nervous systems.

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Figure 1: Notable faces in myasthenia gravis research.
Figure 2: Fetal and adult forms of the acetylcholine receptor.
Figure 3: Pathology of myasthenia gravis.
Figure 4: The importance of divalency in the antibody-induced loss of AChRs.

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Acknowledgements

I would like to thank the colleagues who have helped to shape my views, particularly J. Newsom-Davis FRS, N. Wilcox, B. Lang and D. Beeson. Equally important are the many others around the world who have made important contributions to the topic; I am sorry that I have not been able to do justice to all of their work. My own research on myasthenia gravis over the past 30 years has been generously funded by the Medical Research Council, the Myasthenia Gravis Association, the Muscular Dystrophy Campaign, Action Research and the National Lotteries Charity Board.

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DATABASES

Cancer.gov

lung cancer

thymoma

LocusLink

AChR

C9

GLUR3

IFN-α

IL-12

MuSK

ryanodine receptor

titin

Medscape DrugInfo

azathioprine

physostigmine

OMIM

Hashimoto's thyroiditis

Lambert–Eaton myasthenic syndrome

myasthenia gravis

Rasmussen's encephalitis

FURTHER INFORMATION

Mary Walker

Myasthenia Gravis Foundation Educational Services

Myasthenia Gravis Links

The Myasthenia Gravis Association

Thomas Willis

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Vincent, A. Unravelling the pathogenesis of myasthenia gravis. Nat Rev Immunol 2, 797–804 (2002). https://doi.org/10.1038/nri916

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