Acetylcholine receptors loss and postsynaptic damage in MuSK antibody-positive myasthenia gravis
Acetylcholine receptors loss and postsynaptic damage in MuSK antibody-positive myasthenia gravis
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DOI：
10.1002/ana.20341
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发表时间：
2005-02-01
影响因子：
11.2
通讯作者：
Eguchi, K
中科院分区：
文献类型：
--
作者：
Shiraishi, H;Motomura, M;Eguchi, K
肌肉特异性酪氨酸激酶（MUSK）抗体在一些血清阴性的重症肌无力（MG）患者中发现，但它们如何引起肌无力症状尚不清楚。我们可视化乙酰胆碱受体（AChRs）和补体成分3（C3）肌肉活检从10例日本MG患者与MUSK抗体，与42例AChR抗体。与AChR抗体阳性终板相比，MUSK抗体（Ab）阳性终板的AChR密度没有显著降低，并且在8例MUSK Ab阳性患者中仅2例检测到C3。MUSK抗体似乎不会引起大量AChR损失、补体沉积或形态学损伤。对MUSK功能的影响需要探索。
Muscle-specific tyrosine kinase (MUSK) antibodies are found in some patients with "seronegative" myasthenia gravis (MG), but how they cause myasthenic symptoms is not clear. We visualized acetylcholine receptors (AChRs) and complement component 3 (C3) in muscle biopsies from 10 Japanese MG patients with MUSK antibodies, compared with 42 with AChR antibodies. The AChR density was not significantly decreased in MUSK antibody (Ab)-positive end-plates compared with AChR antibody positive end-plates, and C3 was detected in only two of eight MUSK Ab-positive patients. MUSK antibodies do not appear to cause substantial AChR loss, complement deposition, or morphological damage. Effects on MUSK function need to be explored.