Seronegative generalised myasthenia gravis: clinical features, antibodies, and their targets

Vincent, Angela; Bowen, John M.; Newsom‐Davis, John; McConville, John

doi:10.1016/s1474-4422(03)00306-5

Cited by 199 publications

(164 citation statements)

References 74 publications

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“…These MG patients can be divided into two groups: those with antibodies to muscle-specific kinase (MuSK) [36] and those without AChR and MuSK antibodies [37]. MuSK is essential for AChR clustering at the developing neuromuscular junction and its deficiency may lead to the complete loss of junctional ultrastructure supporting its critical role at the nerve-muscle synapse [38].…”

Section: Autoantibodies In Mgmentioning

confidence: 99%

Effect of complement and its regulation on myasthenia gravis pathogenesis

Kusner¹,

Kaminski²,

Šoltýs³

2008

Expert Review of Clinical Immunology

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Myasthenia gravis (MG) is primarily caused by antibodies directed towards the skeletal muscle acetylcholine receptor, leading to muscle weakness. Although these antibodies may induce compromise of neuromuscular transmission by blocking acetylcholine receptor function or antigenic modulation, the predominant mechanism of injury to the neuromuscular junction is complement-mediated lysis of the postsynaptic membrane. The vast majority of data to support the role of complement derives from experimentally acquired MG (EAMG). In this article, we review studies that demonstrate the central role of complement in EAMG and MG pathogenesis along with the emerging role of complement in T-and B-cell function, as well as the potential for complement inhibitor-based therapy to treat human MG.

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Section: Autoantibodies In Mgmentioning

confidence: 99%

Effect of complement and its regulation on myasthenia gravis pathogenesis

Kusner¹,

Kaminski²,

Šoltýs³

2008

Expert Review of Clinical Immunology

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“…However, MuSK-MG was not identified yet (2001) during these studies (10). In 2003, IgM antibodies were again found in seronegative patients, but not in the MuSK-MG fraction (19). Normally, these IgM autoantibodies are not found during established disease (20).…”

Section: Musk-mg Patients In Vitromentioning

confidence: 86%

Myasthenia gravis: a pathologic approach

Hounjet¹

2016

MARBLE

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Our immune system protects us from all kinds of pathogens our body faces every day.However, the immune system can also initiate an immune response against our own proteins. This is called autoimmunity. Myasthenia gravis (MG) is a rare autoimmune disease in which patients show severe muscle weakness, due to antibody production against proteins of the neuromuscular junction, which is the connection between nerve and muscle. At this moment, three different forms of myasthenia gravis are identified.Muscle-specific tyrosine kinase (MuSK)-MG is a form of MG in which patients produce antibodies, predominantly immunoglobulin (Ig)G4, against MuSK. This paper is a summary of a B cell immortalization project, in which antibodies produced by MuSK-MG patients are investigated. The aim of this study was to sequence and generate antibodies produced by these patients in vitro to learn more about the pathological mechanism of MuSK-MG. Blood-derived B cells of MuSK-MG patients were immortalized by the Epstein Bar Virusand stimulated to grow and produce antibodies in culture. Genes of these antibodies were amplified and analyzed. These amplification products were cloned in vectors and will be transfected in a human embryonic kidney (HEK)-cell line. This study showed that the B cell immortalization method worked and that MuSK-MG patients predominantly produce IgM antibodies against MuSK. This is very striking as literature states that these patients predominantly produce IgG4 against MuSK. It is suggested that this high production of IgM is due to the absence of the heavy chain isotype switch from IgM to IgG. Moreover, as the pathologic mechanism of MuSK-MG is still unknown, IgM may play an important pathological role.

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“…However, detection of serum AChR antibodies is considered to be the diagnostic gold standard. These antibodies are found in 80-85% of patients with generalized MG and in 50-60% of those with ocular MG (22). The diagnosis can also be confirmed by systemic administration of acetylcholinesterase inhibitors, such as neostigmine or edrophonium ("Tensilon test"), followed by an unequivocal improvement in an objectively weak muscle (23).…”

Section: Diagnosismentioning

confidence: 99%

An update on myasthenia gravis, challenging disease for the dental profession

et al. 2015

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Myasthenia gravis is an autoimmune neuromuscular disorder characterized by fluctuating weakness and skeletal muscle fatigue. Clinical signs and symptoms may vary considerably according to the age at presentation, patterns of autoantibodies and associated thymic abnormalities, so that therapeutic options are highly individualized. Facial and oropharyngeal muscle weakness is common at disease onset, and therefore dentists are often the first health professionals to encounter these patients. Myasthenic patients require special consideration and advice in order to ensure optimal and safe dental treatment. Oral manifestations, treatment timing and modality, the choice and effects of drugs and medications, and prevention of myasthenic crisis are all important aspects with which dentists and oral health care providers should be thoroughly acquainted. (J Oral Sci 57, [161][162][163][164][165][166][167][168] 2015)

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Seronegative generalised myasthenia gravis: clinical features, antibodies, and their targets

Cited by 199 publications

References 74 publications

Effect of complement and its regulation on myasthenia gravis pathogenesis

Effect of complement and its regulation on myasthenia gravis pathogenesis

Myasthenia gravis: a pathologic approach

An update on myasthenia gravis, challenging disease for the dental profession

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