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Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity

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.

Publication metadata

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

Year: 2016

Volume: 291

Pages: 82-88

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


DOI: 10.1016/j.jneuroim.2015.12.016

PubMed id: 26857500


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