PMID- 23278586
OWN - NLM
STAT- MEDLINE
DCOM- 20130221
LR  - 20130102
IS  - 1749-6632 (Electronic)
IS  - 0077-8923 (Linking)
VI  - 1275
DP  - 2012 Dec
TI  - Pathogenic IgG4 subclass autoantibodies in MuSK myasthenia gravis.
PG  - 114-22
LID - 10.1111/j.1749-6632.2012.06808.x [doi]
AB  - Autoantibodies against muscle-specific kinase (MuSK), a protein essential for 
      clustering of acetylcholine receptors at the neuromuscular junction (NMJ), are 
      detected in the serum of a proportion of myasthenia gravis (MG) patients. In most 
      MuSK MG patients the anti-MuSK activity resides in the IgG4 subclass, a minor IgG 
      component without very well-defined, but presumably anti-inflammatory, roles in 
      immunity. In recent years, several animal model studies showed that anti-MuSK 
      autoantibodies can cause muscle weakness by directly affecting NMJ function and, 
      therefore, are likely not simply bystander disease markers in MuSK MG patients. 
      In passive transfer mice, we recently provided proof that MuSK MG patient IgG4 is 
      severely myasthenogenic, causing functional defects at NMJs. Against the 
      clinical, serological, and pharmacological background of MuSK MG, here we discuss 
      the MuSK MG animal models generated by our laboratory and others that have been 
      instrumental in elucidating the etiological and pathophysiological roles of 
      anti-MuSK antibodies.
CI  - (c) 2012 New York Academy of Sciences.
FAU - Plomp, Jaap J
AU  - Plomp JJ
AD  - Department of Neurology, Medical Genetics Center, Leiden University Medical 
      Center, Leiden, the Netherlands. j.j.plomp@lumc.nl
FAU - Huijbers, Maartje G
AU  - Huijbers MG
FAU - van der Maarel, Silvere M
AU  - van der Maarel SM
FAU - Verschuuren, Jan J
AU  - Verschuuren JJ
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - United States
TA  - Ann N Y Acad Sci
JT  - Annals of the New York Academy of Sciences
JID - 7506858
RN  - 0 (Autoantibodies)
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  - Animals
MH  - Autoantibodies/*blood
MH  - Disease Models, Animal
MH  - Humans
MH  - Immunoglobulin G/*immunology
MH  - Mice
MH  - Myasthenia Gravis/*immunology/physiopathology
MH  - Receptor Protein-Tyrosine Kinases/*immunology
MH  - Receptors, Cholinergic/*immunology
EDAT- 2013/01/03 06:00
MHDA- 2013/02/22 06:00
CRDT- 2013/01/03 06:00
PHST- 2013/01/03 06:00 [entrez]
PHST- 2013/01/03 06:00 [pubmed]
PHST- 2013/02/22 06:00 [medline]
AID - 10.1111/j.1749-6632.2012.06808.x [doi]
PST - ppublish
SO  - Ann N Y Acad Sci. 2012 Dec;1275:114-22. doi: 10.1111/j.1749-6632.2012.06808.x.