PMID- 32194225 OWN - NLM STAT- MEDLINE DCOM- 20210514 LR - 20210514 IS - 1873-7544 (Electronic) IS - 0306-4522 (Linking) VI - 439 DP - 2020 Jul 15 TI - ANTIBODIES AND RECEPTORS: From Neuromuscular Junction to Central Nervous System. PG - 48-61 LID - S0306-4522(20)30152-4 [pii] LID - 10.1016/j.neuroscience.2020.03.009 [doi] AB - Myasthenia gravis (MG) is a relatively rare neurological disease that is usually associated with antibodies to the acetylcholine receptor (AChR). These antibodies (Abs) cause loss of the AChRs from the neuromuscular junction (NMJ), resulting in muscle weakness that can be life-threatening. Another form of the disease is caused by antibodies to muscle specific kinase (MuSK) that result in impaired AChR clustering and numbers at the NMJ, and may also interfere with presynaptic adaptive mechanisms. Other autoimmune disorders, Lambert Eaton myasthenic syndrome and acquired neuromyotonia, are associated with antibodies to presynaptic voltage-gated calcium and potassium channels respectively. All four conditions can be diagnosed by specific clinical features, electromyography and serum antibody tests, and can be treated effectively by a combination of pharmacological approaches and procedures that reduce the levels of the IgG antibodies. They form the first of a spectrum of diseases in which serum autoantibodies bind to extracellular domains of neuronal proteins throughout the nervous system and lead to constellations of clinical features including paralysis, sensory disturbance and pain, memory loss, seizures, psychiatric disturbance and movement disorders. This review will briefly summarize the ways in which this field has developed, since the 1970s when considerable contributions were made in Ricardo Miledi's laboratory at UCL. CI - Copyright (c) 2020 IBRO. Published by Elsevier Ltd. All rights reserved. FAU - Vincent, Angela AU - Vincent A AD - Nuffield Department of Clinical Neurosciences, University of Oxford, John Radcliffe Hospital, OX3 9DU, UK. Electronic address: Angela.vincent@ndcn.ox.ac.uk. LA - eng PT - Journal Article PT - Review DEP - 20200317 PL - United States TA - Neuroscience JT - Neuroscience JID - 7605074 SB - IM MH - Central Nervous System MH - Humans MH - *Isaacs Syndrome MH - *Lambert-Eaton Myasthenic Syndrome MH - *Myasthenia Gravis MH - Neuromuscular Junction OTO - NOTNLM OT - acetylcholine receptor OT - antibody OT - muscle specific kinase OT - neurological disease OT - neuromuscular junction OT - neuromuscular transmission EDAT- 2020/03/21 06:00 MHDA- 2021/05/15 06:00 CRDT- 2020/03/21 06:00 PHST- 2020/01/08 00:00 [received] PHST- 2020/03/04 00:00 [revised] PHST- 2020/03/05 00:00 [accepted] PHST- 2020/03/21 06:00 [pubmed] PHST- 2021/05/15 06:00 [medline] PHST- 2020/03/21 06:00 [entrez] AID - S0306-4522(20)30152-4 [pii] AID - 10.1016/j.neuroscience.2020.03.009 [doi] PST - ppublish SO - Neuroscience. 2020 Jul 15;439:48-61. doi: 10.1016/j.neuroscience.2020.03.009. Epub 2020 Mar 17.