PMID- 30011414
OWN - NLM
STAT- MEDLINE
DCOM- 20190107
LR  - 20190107
IS  - 1098-9021 (Electronic)
IS  - 0271-8235 (Linking)
VI  - 38
IP  - 3
DP  - 2018 Jun
TI  - Pathogenic Mechanisms and Clinical Correlations in Autoimmune Myasthenic 
      Syndromes.
PG  - 344-354
LID - 10.1055/s-0038-1660500 [doi]
AB  - Autoimmune myasthenic syndromes are antibody-mediated disorders of the 
      neuromuscular junction. Common antigenic targets are the acetylcholine receptor 
      or muscle specific kinase (MuSK) in myasthenia gravis (MG) and the voltage-gated 
      calcium channel in Lambert-Eaton myasthenic syndrome. There is evidence that 
      antibodies directed against other antigens such as low-density lipoprotein 
      receptor-related protein 4 (LRP4) are also involved in MG. The mechanisms by 
      which various antibodies exert their pathogenic effect depend on the IgG subclass 
      and also the epitope location on the antigens. These mechanisms are partly 
      heterogeneous and include antigen degradation, complement activation, direct 
      functional blocking, or disruption of protein-protein interactions. The 
      neuromuscular junction is characterized by a structural and functional plasticity 
      that is able to compensate for some of the neuromuscular junction defects. Here, 
      we discuss the underlying pathogenic mechanisms of the different autoantibodies 
      and correlate them with phenotypic features. The understanding of these elements 
      should help guide the clinical management of patients with autoimmune myasthenic 
      syndromes.
CI  - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
FAU - Cetin, Hakan
AU  - Cetin H
AD  - Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, 
      United Kingdom.
AD  - Department of Neurology, Medical University of Vienna, Vienna, Austria.
FAU - Vincent, Angela
AU  - Vincent A
AD  - Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, 
      United Kingdom.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20180716
PL  - United States
TA  - Semin Neurol
JT  - Seminars in neurology
JID - 8111343
RN  - 0 (Autoantibodies)
RN  - 0 (Immunoglobulin G)
SB  - IM
MH  - Autoantibodies/*immunology
MH  - Humans
MH  - Immunoglobulin G/immunology
MH  - Lambert-Eaton Myasthenic Syndrome/*immunology/*pathology
MH  - Muscle Weakness/immunology/pathology
MH  - Myasthenia Gravis/immunology/pathology
MH  - Neuromuscular Junction/*immunology
COIS- Disclosure The authors report no conflicts of interest in this work.
EDAT- 2018/07/17 06:00
MHDA- 2019/01/08 06:00
CRDT- 2018/07/17 06:00
PHST- 2018/07/17 06:00 [entrez]
PHST- 2018/07/17 06:00 [pubmed]
PHST- 2019/01/08 06:00 [medline]
AID - 10.1055/s-0038-1660500 [doi]
PST - ppublish
SO  - Semin Neurol. 2018 Jun;38(3):344-354. doi: 10.1055/s-0038-1660500. Epub 2018 Jul 
      16.