title: The auto-antigen repertoire in myasthenia gravis.
abstract: Myasthenia Gravis (MG) is an antibody-mediated autoimmune disorder affecting the postsynaptic membrane of the neuromuscular junction (NMJ). MG is characterized by an impaired signal transmission between the motor neuron and the skeletal muscle cell, caused by auto-antibodies directed against NMJ proteins. The auto-antibodies target the nicotinic acetylcholine receptor (nAChR) in about 90% of MG patients. In approximately 5% of MG patients, the muscle specific kinase (MuSK) is the auto-antigen. In the remaining 5% of MG patients, however, antibodies against the nAChR or MuSK are not detectable (idiopathic MG, iMG). Although only the anti-nAChR and anti-MuSK auto-antibodies have been demonstrated to be pathogenic, several other antibodies recognizing self-antigens can also be found in MG patients. Various auto-antibodies associated with thymic abnormalities have been reported, as well as many non-MG-specific auto-antibodies. However, their contribution to the cause, pathology and severity of the disease is still poorly understood. Here, we comprehensively review the reported auto-antibodies in MG patients and discuss their role in the pathology of this autoimmune disease.
authors.affiliation: Division of Neuroscience, School of Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.
authors.forename: Kathleen
authors.initials: K
authors.lastname: Vrolix
authors.forename: Judith
authors.initials: J
authors.lastname: Fraussen
authors.forename: Peter C
authors.initials: PC
authors.lastname: Molenaar
authors.forename: Mario
authors.initials: M
authors.lastname: Losen
authors.forename: Veerle
authors.initials: V
authors.lastname: Somers
authors.forename: Piet
authors.initials: P
authors.lastname: Stinissen
authors.forename: Marc H
authors.initials: MH
authors.lastname: De Baets
authors.forename: Pilar
authors.initials: P
authors.lastname: Martínez-Martínez
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000097:blood / Q000145:classification / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology; D016207:Cytokines / Q000276:immunology; D006801:Humans; D018908:Muscle Weakness / Q000276:immunology; D009157:Myasthenia Gravis / Q000276:immunology* / Q000503:physiopathology; D009469:Neuromuscular Junction / Q000276:immunology / Q000503:physiopathology; D020794:Receptor Protein-Tyrosine Kinases / Q000276:immunology*; D011950:Receptors, Cholinergic / Q000276:immunology*; D011978:Receptors, Nicotinic / Q000276:immunology*; D013950:Thymus Gland / Q000276:immunology / Q000503:physiopathology
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D016207:Cytokines; D011950:Receptors, Cholinergic; D011978:Receptors, Nicotinic; C095714:MUSK protein, human; D020794:Receptor Protein-Tyrosine Kinases