PMID- 31269763
OWN - NLM
STAT- MEDLINE
DCOM- 20191231
LR  - 20211124
IS  - 2073-4409 (Print)
IS  - 2073-4409 (Electronic)
IS  - 2073-4409 (Linking)
VI  - 8
IP  - 7
DP  - 2019 Jul 2
TI  - Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular 
      Architecture.
LID - 10.3390/cells8070671 [doi]
LID - 671
AB  - Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction 
      (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic 
      acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density 
      lipoprotein receptor-related protein 4 (Lrp4), that lead by a range of different 
      pathogenic mechanisms to altered tissue architecture and reduced densities or 
      functionality of AChRs, reduced neuromuscular transmission, and therefore a 
      severe fatigable skeletal muscle weakness. In this review, we give an overview of 
      the history and clinical aspects of MG, with a focus on the structure and 
      function of myasthenic autoantigens at the NMJ and how they are affected by the 
      autoantibodies' pathogenic mechanisms. Furthermore, we give a short overview of 
      the cells that are implicated in the production of the autoantibodies and briefly 
      discuss diagnostic challenges and treatment strategies.
FAU - Koneczny, Inga
AU  - Koneczny I
AD  - Institute of Neurology, Medical University of Vienna, 1090 Vienna, Austria. 
      inga.koneczny@meduniwien.ac.at.
FAU - Herbst, Ruth
AU  - Herbst R
AD  - Center for Pathophysiology, Infectiology and Immunology, Medical University of 
      Vienna, 1090 Vienna, Austria. ruth.herbst@meduniwien.ac.at.
LA  - eng
GR  - P 28485/FWF_/Austrian Science Fund FWF/Austria
GR  - T 996/FWF_/Austrian Science Fund FWF/Austria
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20190702
PL  - Switzerland
TA  - Cells
JT  - Cells
JID - 101600052
RN  - 0 (Agrin)
RN  - 0 (Autoantibodies)
RN  - 0 (Autoantigens)
RN  - 0 (LDL-Receptor Related Proteins)
RN  - 0 (Receptors, Cholinergic)
RN  - 0 (Receptors, Nicotinic)
RN  - EC 2.7.10.1 (MUSK protein, human)
RN  - EC 2.7.10.1 (MuSK protein, mouse)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Agrin/immunology/metabolism
MH  - Animals
MH  - Autoantibodies/*immunology
MH  - Autoantigens/*immunology/metabolism
MH  - Humans
MH  - LDL-Receptor Related Proteins/immunology/metabolism
MH  - Muscle, Skeletal/immunology/*pathology/ultrastructure
MH  - Myasthenia Gravis/*immunology/pathology
MH  - Neuromuscular Junction/immunology/*pathology/ultrastructure
MH  - Receptor Protein-Tyrosine Kinases/immunology/metabolism
MH  - Receptors, Cholinergic/immunology/metabolism
MH  - Receptors, Nicotinic/immunology/metabolism
PMC - PMC6678492
OTO - NOTNLM
OT  - AChR
OT  - Agrin
OT  - Lrp4
OT  - MuSK
OT  - autoantibodies
OT  - autoimmunity
OT  - history
OT  - immunopathogenesis
OT  - myasthenia gravis
OT  - neuromuscular junction
COIS- The authors declare no conflict of interest.
EDAT- 2019/07/05 06:00
MHDA- 2019/07/05 06:01
PMCR- 2019/07/01
CRDT- 2019/07/05 06:00
PHST- 2019/06/07 00:00 [received]
PHST- 2019/06/26 00:00 [revised]
PHST- 2019/06/28 00:00 [accepted]
PHST- 2019/07/05 06:00 [entrez]
PHST- 2019/07/05 06:00 [pubmed]
PHST- 2019/07/05 06:01 [medline]
PHST- 2019/07/01 00:00 [pmc-release]
AID - cells8070671 [pii]
AID - cells-08-00671 [pii]
AID - 10.3390/cells8070671 [doi]
PST - epublish
SO  - Cells. 2019 Jul 2;8(7):671. doi: 10.3390/cells8070671.