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MuSK myasthenia gravis IgG4 disrupts the interaction of LRP4 with MuSK but both IgG4 and IgG1-3 can disperse preformed agrin-independent AChR clusters

Abstract:

A variable proportion of patients with generalized myasthenia gravis (MG) have autoantibodies to muscle specific tyrosine kinase (MuSK). During development agrin, released from the motor nerve, interacts with low density lipoprotein receptor-related protein-4 (LRP4), which then binds to MuSK; MuSK interaction with the intracellular protein Dok7 results in clustering of the acetylcholine receptors (AChRs) on the postsynaptic membrane. In mature muscle, MuSK helps maintain the high density of A...

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Publication status:
Published
Peer review status:
Peer reviewed

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Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Clinical Neurosciences
Role:
Author
More by this author
Institution:
University of Oxford
Division:
MSD
Department:
Clinical Neurosciences
Role:
Author
More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Clinical Neurosciences
Role:
Author
More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Clinical Neurosciences
Role:
Author
More by this author
Institution:
University of Oxford
Division:
Medical Sciences Division
Department:
Clinical Neurosciences
Role:
Author
More from this funder
Name:
Wellcome Trust
Grant:
084655
More from this funder
Name:
Medical Research Council
Grant:
G0701521
Publisher:
PLoS
Journal:
PLoS ONE More from this journal
Volume:
8
Issue:
11
Pages:
Article:e80695
Publication date:
2013-11-07
Acceptance date:
2013-10-13
DOI:
EISSN:
1932-6203
Pmid:
24244707
Language:
English
Keywords:
Pubs id:
pubs:440150
UUID:
uuid:05cd37e3-f384-421f-8f5e-1581a28433ca
Local pid:
pubs:440150
Source identifiers:
440150
Deposit date:
2018-01-10

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