PMID- 20380581 OWN - NLM STAT- MEDLINE DCOM- 20101130 LR - 20100819 IS - 1607-842X (Electronic) IS - 0891-6934 (Linking) VI - 43 IP - 5-6 DP - 2010 Aug TI - The auto-antigen repertoire in myasthenia gravis. PG - 380-400 LID - 10.3109/08916930903518073 [doi] AB - 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. FAU - Vrolix, Kathleen AU - Vrolix K AD - Division of Neuroscience, School of Mental Health and Neuroscience, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands. FAU - Fraussen, Judith AU - Fraussen J FAU - Molenaar, Peter C AU - Molenaar PC FAU - Losen, Mario AU - Losen M FAU - Somers, Veerle AU - Somers V FAU - Stinissen, Piet AU - Stinissen P FAU - De Baets, Marc H AU - De Baets MH FAU - Martinez-Martinez, Pilar AU - Martinez-Martinez P LA - eng PT - Journal Article PT - Review PL - England TA - Autoimmunity JT - Autoimmunity JID - 8900070 RN - 0 (Autoantibodies) RN - 0 (Autoantigens) RN - 0 (Cytokines) RN - 0 (Receptors, Cholinergic) RN - 0 (Receptors, Nicotinic) RN - EC 2.7.10.1 (MUSK protein, human) RN - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases) SB - IM MH - Animals MH - Autoantibodies/blood/classification/*immunology MH - Autoantigens/*immunology MH - Autoimmune Diseases/immunology MH - Cytokines/immunology MH - Humans MH - Muscle Weakness/immunology MH - Myasthenia Gravis/*immunology/physiopathology MH - Neuromuscular Junction/immunology/physiopathology MH - Receptor Protein-Tyrosine Kinases/*immunology MH - Receptors, Cholinergic/*immunology MH - Receptors, Nicotinic/*immunology MH - Thymus Gland/immunology/physiopathology EDAT- 2010/04/13 06:00 MHDA- 2010/12/14 06:00 CRDT- 2010/04/13 06:00 PHST- 2010/04/13 06:00 [entrez] PHST- 2010/04/13 06:00 [pubmed] PHST- 2010/12/14 06:00 [medline] AID - 10.3109/08916930903518073 [doi] PST - ppublish SO - Autoimmunity. 2010 Aug;43(5-6):380-400. doi: 10.3109/08916930903518073.