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Passive transfer models of myasthenia gravis with muscle-specific kinase antibodies.
Verschuuren JJGM1, Plomp JJ1, Burden SJ2, Zhang W2, Fillié-Grijpma YE1, Stienstra-van Es IE1, Niks EH1, Losen M3, van der Maarel SM1, Huijbers MG1.
Ann N Y Acad Sci. 2018 Feb;1413(1):111-118. doi: 10.1111/nyas.13543. Epub 2018 Jan 21.
Abstract
Myasthenia gravis (MG) with antibodies to muscle-specific kinase (MuSK) is characterized by fluctuating fatigable weakness.
In MuSK MG, involvement of bulbar muscles, neck, and shoulder and respiratory weakness are more prominent than in acetylcholine receptor (AChR) MG.
MuSK autoantibodies are mainly of the IgG4 subclass, and as such are unable to activate complement, have low affinity for Fc receptors, and are functionally monovalent.
Therefore, the pathogenicity of IgG4 MuSK autoantibodies was initially questioned.
A broad collection of in vitro active immunization and passive transfer models has been developed that have shed light on the pathogenicity of MuSK autoantibodies.
Passive transfer studies with purified IgG4 from MuSK MG patients confirmed that IgG4 is sufficient to reproduce clear clinical, electrophysiological, and histological signs of myasthenia.
In vitro experiments revealed that MuSK IgG4 autoantibodies preferably bind the first Ig-like domain of MuSK, correlate with disease severity, and interfere with the association between MuSK and low-density lipoprotein receptor-related protein 4 and collagen Q.
Some patients have additional IgG1 MuSK autoantibodies, but their role in the disease is unclear.
Altogether, this provides a rationale for epitope-specific or IgG4-specific treatment strategies for MuSK MG and emphasizes the importance of the development of different experimental models.
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