PMID- 32457737
OWN - NLM
STAT- MEDLINE
DCOM- 20210325
LR  - 20210810
IS  - 1664-3224 (Electronic)
IS  - 1664-3224 (Linking)
VI  - 11
DP  - 2020
TI  - Muscle-Specific Kinase Myasthenia Gravis.
PG  - 707
LID - 10.3389/fimmu.2020.00707 [doi]
LID - 707
AB  - Thirty to fifty percent of patients with acetylcholine receptor (AChR) antibody 
      (Ab)-negative myasthenia gravis (MG) have Abs to muscle specific kinase (MuSK) 
      and are referred to as having MuSK-MG. MuSK is a 100 kD single-pass post-synaptic 
      transmembrane receptor tyrosine kinase crucial to the development and maintenance 
      of the neuromuscular junction. The Abs in MuSK-MG are predominantly of the IgG4 
      immunoglobulin subclass. MuSK-MG differs from AChR-MG, in exhibiting more focal 
      muscle involvement, including neck, shoulder, facial and bulbar-innervated 
      muscles, as well as wasting of the involved muscles. MuSK-MG is highly associated 
      with the HLA DR14-DQ5 haplotype and occurs predominantly in females with onset in 
      the fourth decade of life. Some of the standard treatments of AChR-MG have been 
      found to have limited effectiveness in MuSK-MG, including thymectomy and 
      cholinesterase inhibitors. Therefore, current treatment involves 
      immunosuppression, primarily by corticosteroids. In addition, patients respond 
      especially well to B cell depletion agents, e.g., rituximab, with long-term 
      remissions. Future treatments will likely derive from the ongoing analysis of the 
      pathogenic mechanisms underlying this disease, including histologic and 
      physiologic studies of the neuromuscular junction in patients as well as 
      information derived from the development and study of animal models of the 
      disease.
CI  - Copyright (c) 2020 Borges and Richman.
FAU - Borges, Lucia S
AU  - Borges LS
AD  - Department of Neurology, University of California, Davis, Davis, CA, United 
      States.
FAU - Richman, David P
AU  - Richman DP
AD  - Department of Neurology, University of California, Davis, Davis, CA, United 
      States.
LA  - eng
GR  - R21 NS104516/NS/NINDS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20200508
PL  - Switzerland
TA  - Front Immunol
JT  - Frontiers in immunology
JID - 101560960
RN  - 0 (Adrenal Cortex Hormones)
RN  - 0 (HLA-DR Serological Subtypes)
RN  - 0 (HLA-DR14)
RN  - 0 (Immunoglobulin G)
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
MH  - Adrenal Cortex Hormones/therapeutic use
MH  - Animals
MH  - Female
MH  - HLA-DR Serological Subtypes/genetics
MH  - Haplotypes
MH  - Humans
MH  - Immunoglobulin G/immunology
MH  - Mice
MH  - Muscles/*pathology
MH  - Myasthenia Gravis/drug therapy/*enzymology/genetics/*pathology
MH  - Receptor Protein-Tyrosine Kinases/genetics
MH  - Receptors, Cholinergic/genetics
PMC - PMC7225350
OTO - NOTNLM
OT  - animal models
OT  - muscle specific kinase
OT  - myasthenia gravis
OT  - neuromuscular junction
OT  - pathogenesis
OT  - review
OT  - treatment
EDAT- 2020/05/28 06:00
MHDA- 2021/03/26 06:00
PMCR- 2020/01/01
CRDT- 2020/05/28 06:00
PHST- 2019/11/11 00:00 [received]
PHST- 2020/03/30 00:00 [accepted]
PHST- 2020/05/28 06:00 [entrez]
PHST- 2020/05/28 06:00 [pubmed]
PHST- 2021/03/26 06:00 [medline]
PHST- 2020/01/01 00:00 [pmc-release]
AID - 10.3389/fimmu.2020.00707 [doi]
PST - epublish
SO  - Front Immunol. 2020 May 8;11:707. doi: 10.3389/fimmu.2020.00707. eCollection 
      2020.