@article{069467365a6a4a1d847ab3908ae7e3e2,
title = "Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity",
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.",
keywords = "Epitope mapping, IgG4, MuSK, Myasthenia gravis, Neuromuscular junction",
author = "Huijbers, {Maartje G.} and Vink, {Anna Fleur D.} and Niks, {Erik H.} and Westhuis, {Ruben H.} and {van Zwet}, {Erik W.} and {de Meel}, {Robert H.} and Ricardo Rojas-Garc{\'i}a and Jordi D{\'i}az-Manera and Kuks, {Jan B.} and Rinse Klooster and Kirsten Straasheijm and Amelia Evoli and Isabel Illa and {van der Maarel}, {Silv{\`e}re M.} and Verschuuren, {Jan J.}",
note = "Funding Information: Prof. van der Maarel is consultant for aTyr. Prof. van der Maarel is co-inventor on patent applications in the field of FSHD. His research is supported by funds from the National Institutes of Health, the Prinses Beatrix Spierfonds, the European Union, Spieren voor Spieren, FSHD Global Research Foundation, and from Argen-X Funding Information: We would like to thank Ingrid Hegeman for providing us with excellent support in serum maintenance. This study was supported by funding of the Prinses Beatrix Spierfonds ( W.OR09-19 ) and the Association Fran{\c c}aise contre les Myopathies ( 15363 ). Funding Information: Prof. Illa has received research funds from Grifols and received speaking fees and travel grants from Grifols, Genzyme and Pfizer. Prof. Illa provided expert testimony to Alexion, UCB and Grifols. Funding Information: Dr. Niks reports grants from Duchenne Parent Project, ZonMW, and AFM outside the submitted work. He is or has been involved in clinical trials on Duchenne muscular dystrophy with Prosensa, GSK, Biomarin, Lilly and Santhera and provided scientific advice for Summit Therapeutics. He has not received personal payments. Funding Information: Dr. Rojas receive consultant fees from serving on the scientific advisory board for Pfizer, is funded by Fondo de Investigacion Sanitaria FIS PI13/00772 and received research support from Fundacio La Marato de TV3. Publisher Copyright: {\textcopyright} 2016 Elsevier B.V.",
year = "2016",
month = feb,
day = "15",
doi = "10.1016/j.jneuroim.2015.12.016",
language = "English",
volume = "291",
pages = "82--88",
journal = "Journal of Neuroimmunology",
issn = "0165-5728",
publisher = "Elsevier",
}