MuSK型重症肌无力的纵向表位作图：对疾病严重程度的影响
Longitudinal epitope mapping in MuSK myasthenia gravis: implications for disease severity.
作者信息
Huijbers Maartje G, Vink Anna-Fleur D, Niks Erik H, Westhuis Ruben H, van Zwet Erik W, de Meel Robert H, Rojas-García Ricardo, Díaz-Manera Jordi, Kuks Jan B, Klooster Rinse, Straasheijm Kirsten, Evoli Amelia, Illa Isabel, van der Maarel Silvère M, Verschuuren Jan J
机构信息
Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands; Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands.
Department of Human Genetics, Leiden University Medical Centre, Leiden, The Netherlands.
出版信息
J Neuroimmunol. 2016 Feb 15;291:82-8. doi: 10.1016/j.jneuroim.2015.12.016. Epub 2016 Jan 5.
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.
肌肉特异性激酶（MuSK）型重症肌无力（MG）中的肌无力主要由IgG4抗体引起，这些抗体阻断MuSK信号传导并破坏神经肌肉接头的稳定性。我们确定了MuSK IgG4抗体的结合模式在整个疾病过程中是否发生变化（“表位扩展”），以及是否影响疾病严重程度或治疗反应性。我们用酶联免疫吸附测定法（ELISA）绘制了来自三个独立队列的53例独特的MuSK MG患者的255份纵向血清样本的MuSK表位图谱。针对MuSK免疫球蛋白样-1结构域的抗体决定疾病严重程度。该结构域之外的表位扩展对疾病严重程度或溴吡斯的明反应性均无影响。这为表位特异性治疗策略提供了理论依据。
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