PMID- 18646570
OWN - NLM
STAT- MEDLINE
DCOM- 20080826
LR  - 20211020
IS  - 1128-2460 (Print)
IS  - 1128-2460 (Linking)
VI  - 26
IP  - 3
DP  - 2007 Dec
TI  - Myasthenia gravis induced by autoantibodies against MuSK.
PG  - 185-91
AB  - Myasthenia gravis (MG) is caused by the failure of neuromuscular transmission 
      mediated by autoantibodies. That is, the binding of autoantibodies to 
      postsynaptic membranes in neuromuscular junctions (NMJ) results in weakening of 
      the ocular, bulbar and limb muscles and produces the characteristic syndrome of 
      MG. This relatively rare disease serves as a model not only for study of the 
      pathogenesis and treatment of all autoimmune disorders but also for understanding 
      the basic mechanisms of neuromuscular transmission at the NMJ. About 80 to 85% of 
      patients with MG have autoantibodies against acetylcholine receptors (AChR). 
      Although a number of studies have shown the possible existence of other 
      autoantibodies in the remaining approximately 20% of MG patients, the responsible 
      autoantigens have remained elusive. However, antibodies against muscle-specific 
      kinase (MuSK) have been found in 30% of MG patients without AChR antibodies. 
      MuSK, a tyrosine kinase receptor, is required for the development of NMJ's 
      postsynaptic membranes. Still, the pathogenicity of MuSK antibodies as a cause of 
      muscle weakness in patients with MG remains a matter of dispute, because the 
      experimental autoimmune MG caused by MuSK antibodies in animals was absent. Here 
      we describe recent progress toward understanding the pathogenic role of MuSK 
      antibodies in the decline of muscle strength that typifies MG.
FAU - Shigemoto, K
AU  - Shigemoto K
AD  - Research Team for Molecular Biomarkers, Tokyo Metropolitan Institute of 
      Gerontology, Tokyo, Japan. kazshige@tmig.or.jp
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PL  - Italy
TA  - Acta Myol
JT  - Acta myologica : myopathies and cardiomyopathies : official journal of the 
      Mediterranean Society of Myology
JID - 9811169
RN  - 0 (Autoantibodies)
RN  - EC 2.7.10.1 (MuSK protein, mouse)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Animals
MH  - Autoantibodies/*immunology
MH  - Autoimmunity/*immunology
MH  - Disease Models, Animal
MH  - Humans
MH  - Myasthenia Gravis/*immunology
MH  - Receptor Protein-Tyrosine Kinases/*immunology
PMC - PMC2949310
EDAT- 2008/07/24 09:00
MHDA- 2008/08/30 09:00
PMCR- 2007/12/01
CRDT- 2008/07/24 09:00
PHST- 2008/07/24 09:00 [pubmed]
PHST- 2008/08/30 09:00 [medline]
PHST- 2008/07/24 09:00 [entrez]
PHST- 2007/12/01 00:00 [pmc-release]
PST - ppublish
SO  - Acta Myol. 2007 Dec;26(3):185-91.