PMID- 19428298
OWN - NLM
STAT- MEDLINE
DCOM- 20090804
LR  - 20211028
IS  - 1471-4892 (Print)
IS  - 1471-4892 (Linking)
VI  - 9
IP  - 3
DP  - 2009 Jun
TI  - Autoimmune disorders of the neuromuscular junction.
PG  - 336-40
LID - 10.1016/j.coph.2009.04.005 [doi]
AB  - The neuromuscular junction lies beyond the protection of the blood-brain barrier 
      and is particularly vulnerable to antibody-mediated attack. In myasthenia gravis, 
      the expression of acetylcholine receptors (AChRs) in the thymus is under the 
      control of the autoimmune regulator protein (AIRE), and polymorphisms in the AChR 
      correlate with early onset of disease. In some 'AChR seronegative' patients, 
      thymic abnormalities associated with complement-activating antibodies binding 
      only clustered AChRs have been demonstrated, and in others anti-muscle-specific 
      kinase (MuSK) antibodies that show pathogenic effects in vivo. In Guillain-Barre 
      syndrome, newly described antibodies bind to complex gangliosides. General 
      immunosuppression is still the main treatment, but novel treatments that reduce 
      complement-mediated damage or inhibit the binding of pathogenic antibodies are 
      beginning to look promising.
FAU - Lang, Bethan
AU  - Lang B
AD  - Department of Clinical Neurology, University of Oxford, Oxford OX3 9DS, UK. 
      blang@hammer.imm.ox.ac.uk
FAU - Vincent, Angela
AU  - Vincent A
LA  - eng
GR  - 084655/WT_/Wellcome Trust/United Kingdom
PT  - Journal Article
PT  - Review
DEP - 20090508
PL  - England
TA  - Curr Opin Pharmacol
JT  - Current opinion in pharmacology
JID - 100966133
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Animals
MH  - Disease Models, Animal
MH  - Guillain-Barre Syndrome/immunology/therapy
MH  - Humans
MH  - Immunosuppressive Agents/*therapeutic use
MH  - Immunotherapy/*methods
MH  - Isaacs Syndrome/immunology/therapy
MH  - Lambert-Eaton Myasthenic Syndrome/immunology/therapy
MH  - Myasthenia Gravis/immunology/therapy
MH  - Neuromuscular Junction Diseases/immunology/*therapy
RF  - 38
EDAT- 2009/05/12 09:00
MHDA- 2009/08/06 09:00
CRDT- 2009/05/12 09:00
PHST- 2009/01/27 00:00 [received]
PHST- 2009/03/23 00:00 [revised]
PHST- 2009/04/14 00:00 [accepted]
PHST- 2009/05/12 09:00 [entrez]
PHST- 2009/05/12 09:00 [pubmed]
PHST- 2009/08/06 09:00 [medline]
AID - S1471-4892(09)00046-0 [pii]
AID - 10.1016/j.coph.2009.04.005 [doi]
PST - ppublish
SO  - Curr Opin Pharmacol. 2009 Jun;9(3):336-40. doi: 10.1016/j.coph.2009.04.005. Epub 
      2009 May 8.