| Literature DB >> 26857500 |
Maartje G Huijbers1, Anna-Fleur D Vink2, Erik H Niks3, Ruben H Westhuis3, Erik W van Zwet4, Robert H de Meel3, Ricardo Rojas-García5, Jordi Díaz-Manera5, Jan B Kuks6, Rinse Klooster2, Kirsten Straasheijm2, Amelia Evoli7, Isabel Illa5, Silvère M van der Maarel2, Jan J Verschuuren3.
Abstract
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.Entities:
Keywords: Epitope mapping; IgG4; MuSK; Myasthenia gravis; Neuromuscular junction
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Year: 2016 PMID： 26857500 DOI： 10.1016/j.jneuroim.2015.12.016
Source DB: PubMed Journal: J Neuroimmunol ISSN： 0165-5728 Impact factor: 3.478