Shiraishi Hirokazu, Motomura Masakatsu, Yoshimura Toshiro, Fukudome Takayasu, Fukuda Taku, Nakao Yoko, Tsujihata Mitsuhiro, Vincent Angela, Eguchi Katsumi
The First Department of Internal Medicine, Graduate School of Biomedical Sciences, Nagasaki, Japan.
Ann Neurol. 2005 Feb;57(2):289-93. doi: 10.1002/ana.20341.
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.
在一些“血清阴性”重症肌无力(MG)患者中可检测到肌肉特异性酪氨酸激酶(MuSK)抗体,但它们如何导致肌无力症状尚不清楚。我们对10例患有MuSK抗体的日本MG患者的肌肉活检组织中的乙酰胆碱受体(AChR)和补体成分3(C3)进行了可视化分析,并与42例患有AChR抗体的患者进行了比较。与AChR抗体阳性的终板相比,MuSK抗体(Ab)阳性终板中的AChR密度没有显著降低,并且在8例MuSK Ab阳性患者中只有2例检测到C3。MuSK抗体似乎不会导致大量AChR丢失、补体沉积或形态学损伤。需要探索其对MuSK功能的影响。