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Lookup NU author(s): Professor Jordi Diaz ManeraORCiD
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© 2016 Elsevier B.V. Muscle weakness in MuSK myasthenia gravis (MG) is caused predominantly by IgG4 antibodies which block MuSK signalling and destabilize neuromuscular junctions. We determined whether the binding pattern of MuSK IgG4 antibodies change throughout the disease course ("epitope spreading"), and affect disease severity or treatment responsiveness.We mapped the MuSK epitopes of 255 longitudinal serum samples of 53 unique MuSK MG patients from three independent cohorts with ELISA.Antibodies against the MuSK Iglike-1 domain determine disease severity. Epitope spreading outside this domain did not contribute to disease severity nor to pyridostigmine responsiveness. This provides a rationale for epitope specific treatment strategies.
Author(s): Huijbers MG, Vink A-FD, Niks EH, Westhuis RH, van Zwet EW, de Meel RH, Rojas-Garcia R, Diaz-Manera J, Kuks JB, Klooster R, Straasheijm K, Evoli A, Illa I, van der Maarel SM, Verschuuren JJ
Publication type: Article
Publication status: Published
Journal: Journal of Neuroimmunology
Print publication date: 15/02/2016
Online publication date: 05/01/2016
Acceptance date: 31/12/2015
ISSN (print): 0165-5728
ISSN (electronic): 1872-8421
Publisher: Elsevier BV
PubMed id: 26857500
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