PMID- 29377162 OWN - NLM STAT- MEDLINE DCOM- 20190311 LR - 20211124 IS - 1749-6632 (Electronic) IS - 0077-8923 (Linking) VI - 1413 IP - 1 DP - 2018 Feb TI - Serological and experimental studies in different forms of myasthenia gravis. PG - 143-153 LID - 10.1111/nyas.13592 [doi] AB - 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. CI - (c) 2018 New York Academy of Sciences. FAU - Vincent, Angela AU - Vincent A AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Huda, Saif AU - Huda S AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Cao, Michelangelo AU - Cao M AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Cetin, Hakan AU - Cetin H AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Koneczny, Inga AU - Koneczny I AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Rodriguez Cruz, Pedro M AU - Rodriguez Cruz PM AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Jacobson, Leslie AU - Jacobson L AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Viegas, Stuart AU - Viegas S AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Jacob, Saiju AU - Jacob S AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Woodhall, Mark AU - Woodhall M AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Nagaishi, Akiko AU - Nagaishi A AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Maniaol, Angelina AU - Maniaol A AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Damato, Valentina AU - Damato V AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Leite, M Isabel AU - Leite MI AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Cossins, Judith AU - Cossins J AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Webster, Richard AU - Webster R AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Palace, Jacqueline AU - Palace J AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. FAU - Beeson, David AU - Beeson D AD - Neuroimmunology Group, Nuffield Department of Clinical Neurosciences, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK. LA - eng GR - J 3545/FWF_/Austrian Science Fund FWF/Austria GR - MR/M006824/1/MRC_/Medical Research Council/United Kingdom GR - T 996/FWF_/Austrian Science Fund FWF/Austria PT - Journal Article PT - Review DEP - 20180129 PL - United States TA - Ann N Y Acad Sci JT - Annals of the New York Academy of Sciences JID - 7506858 RN - 0 (Agrin) RN - 0 (Autoantibodies) RN - 0 (Immunoglobulin G) RN - 0 (KCNA4 protein, human) RN - 0 (Kv1.4 Potassium Channel) RN - 0 (LDL-Receptor Related Proteins) RN - 0 (LRP4 protein, human) RN - 0 (Receptors, Cholinergic) RN - EC 2.7.10.1 (MUSK protein, human) RN - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases) SB - IM EIN - Ann N Y Acad Sci. 2018 Apr;1417(1):130. PMID: 29717491 MH - Agrin/immunology MH - Autoantibodies/*immunology MH - Humans MH - Immunoglobulin G/immunology MH - Kv1.4 Potassium Channel/immunology MH - LDL-Receptor Related Proteins/immunology MH - Myasthenia Gravis/classification/*immunology MH - Receptor Protein-Tyrosine Kinases/*immunology MH - Receptors, Cholinergic/*immunology OTO - NOTNLM OT - active and passive immunization OT - clustered acetylcholine receptor OT - low-density lipoprotein receptor-related protein 4 OT - muscle-specific kinase OT - myasthenia gravis EDAT- 2018/01/30 06:00 MHDA- 2019/03/12 06:00 CRDT- 2018/01/30 06:00 PHST- 2017/10/02 00:00 [received] PHST- 2017/12/11 00:00 [revised] PHST- 2017/12/12 00:00 [accepted] PHST- 2018/01/30 06:00 [pubmed] PHST- 2019/03/12 06:00 [medline] PHST- 2018/01/30 06:00 [entrez] AID - 10.1111/nyas.13592 [doi] PST - ppublish SO - Ann N Y Acad Sci. 2018 Feb;1413(1):143-153. doi: 10.1111/nyas.13592. Epub 2018 Jan 29.