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.
journal_name
J Neuroimmunoljournal_title
Journal of neuroimmunologyauthors
Huijbers MG,Vink AF,Niks EH,Westhuis RH,van Zwet EW,de Meel RH,Rojas-García R,Díaz-Manera J,Kuks JB,Klooster R,Straasheijm K,Evoli A,Illa I,van der Maarel SM,Verschuuren JJdoi
10.1016/j.jneuroim.2015.12.016subject
Has Abstractpub_date
2016-02-15 00:00:00pages
82-8eissn
0165-5728issn
1872-8421pii
S0165-5728(15)30109-0journal_volume
291pub_type
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