臨床神経学

第48回日本神経学会総会

<シンポジウム3-1>神経疾患と自己抗体
神経筋接合部疾患における抗MuSK抗体と病態機序

丸山 直記1), 重本 和宏2)

1)東京都老人総合研究所〔〒173-0015 板橋区栄町35-2〕
2)愛媛大学医学部
現 東京都老人総合研究所

Antibodies to acetylcholine receptor (AChR) are major cause of the human autoimmune disease, myasthenia gravis (MG). Additionally, autoantibodies against Muscle-specific kinase (MuSK) were found in a proportion of patients with generalized MG. After the identification of MuSK antibodies in MG patients, laboratory test for measuring antibodies to MuSK is now required to confirm the diagnosis of MG and the clinical treatment as well as AChR antibodies. MuSK is critical to the clustering of AChR and plays multiple roles at neuromuscular junctions (NMJ). However, it has been dispute concerning the pathogenicity of MuSK antibodies in muscle weakness of MG, as the experimental autoimmune MG caused by MuSK antibodies was absent. Here we describe the recent progress to understand the pathogenic roles of MuSK antibodies in muscle weakness of experimental animals induced by MuSK protein.

(臨床神経, 47:842−844, 2007)
key words:重症筋無力症, 筋特異的カイネース, 神経筋接合部, 自己抗体

(受付日:2007年5月16日)