Morphological effects of myasthenia gravis patient sera on human muscle cells

Myasthenia gravis (MG) is caused primarily by autoantibodies against the nicotinic acetylcholine receptor (AChR), but autoantibodies to other muscle proteins may be present. Many of these proteins have structural or signalling functions, the disruption of which may affect muscle cell morphology or v...

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Bibliographic Details
Published in:Muscle & nerve 2006-01, Vol.33 (1), p.93-103
Main Authors: Luckman, Steven Paul, Skeie, Geir Olve, Helgeland, Geir, Gilhus, Nils Erik
Format: Article
Language:English
Subjects:
acetylcholine receptor
Autoantibodies - blood
autoantibody
Biological and medical sciences
Cell Death
cytotoxicity
Diseases of striated muscles. Neuromuscular diseases
Fundamental and applied biological sciences. Psychology
Humans
Immune Sera - toxicity
Medical sciences
morphology
muscle cell culture
Muscle Cells - drug effects
Muscle Cells - pathology
myasthenia gravis
Myasthenia Gravis - blood
Myasthenia Gravis - immunology
Myasthenia Gravis - pathology
Neurology
Striated muscle. Tendons
Vertebrates: osteoarticular system, musculoskeletal system
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Summary:Myasthenia gravis (MG) is caused primarily by autoantibodies against the nicotinic acetylcholine receptor (AChR), but autoantibodies to other muscle proteins may be present. Many of these proteins have structural or signalling functions, the disruption of which may affect muscle cell morphology or viability. In order to investigate the role of such autoantibodies in MG, we examined the effect of MG patient sera, of different autoantibody composition and obtained at different stages of disease severity, on primary human muscle cells. Sera from MG patients induced changes in cell morphology from typical elongated cells to an irregular phenotype, caused the formation of inclusion bodies and intracellular vesicles, and led to a disordered arrangement of actin microfilaments. Sera from the most severely affected patients also induced cell death, which did not occur via classic apoptosis. The effects were not complement‐mediated and were dose‐ and time‐dependent. As the effects observed in the cell culture system correlated with disease severity, a greater understanding of the individual factors responsible for these effects may improve our understanding of MG pathogenesis and be of value in the assessment of disease in individual patients. Muscle Nerve, 2005
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.20443