期刊
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
卷 1413, 期 1, 页码 143-153出版社
WILEY
DOI: 10.1111/nyas.13592
关键词
myasthenia gravis; muscle-specific kinase; clustered acetylcholine receptor; low-density lipoprotein receptor-related protein 4; active and passive immunization
资金
- Austrian Science Fund FWF [T 996, J 3545] Funding Source: Medline
- Medical Research Council [MR/M006824/1] Funding Source: Medline
- Austrian Science Fund (FWF) [J 3545] Funding Source: researchfish
- Medical Research Council [MR/M006824/1] Funding Source: researchfish
- MRC [MR/M006824/1] Funding Source: UKRI
Antibodies to the acetylcholine receptor (AChR) have been recognized for over 40 years and have been important in the diagnosis of myasthenia gravis (MG), and its recognition in patients of different ages and thymic pathologies. The 10-20% of patients who do not have AChR antibodies are now known to comprise different subgroups, the most commonly reported of which is patients with antibodies to muscle-specific kinase (MuSK). The use of cell-based assays has extended the repertoire of antibody tests to clustered AChRs, low-density lipoprotein receptor-related protein 4, and agrin. Autoantibodies against intracellular targets, namely cortactin, titin, and ryanodine receptor (the latter two being associated with the presence of thymoma), may also be helpful as biomarkers in some patients. IgG4 MuSK antibodies are clearly pathogenic, but the coexisting IgG1, IgG2, and IgG3 antibodies, collectively, have effects that question the dominance of IgG4 as the sole pathologic factor in MuSK MG. After a brief historical review, we define the different subgroups and summarize the antibody characteristics. Experiments to demonstrate the in vitro and in vivo pathogenic roles of MuSK antibodies are discussed.
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