Analysis of the aquaporin expression in skeletal muscles with neuromuscular diseases.

神经肌肉疾病骨骼肌中水通道蛋白表达的分析。

• 批准号: 20591020
• 负责人: SHIMIZU Jun
• 金额: $ 3万
• 依托单位: The University of Tokyo
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2008
• 资助国家: 日本
• 起止时间: 2008 至 2010
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-20591020/
• 关键词: 神経筋疾患; aquaporin 4; aquaporin; 筋線維

Aquaporin 4 (AQP4) is known to be important not only in controlling water balance of many cell types but also serve as an autoimmune target of neuromyelitis optica. In this project, we studied AQP4 expression of skeletal muscle fibers in various neuromusclular diseases including inflammatory myopathies. In immunohistochemistry, in addition to regenerating muscle fibers and denervated muscle fibers irrespective of disease, many non necrotic fibers in inflammatory myopathies showed the decrease of AQP4 expression. In the analysis of the factors which correlates to the decrease of AQP4 expression, the deposit of membrane attack complex (C5b9) in muscle tissues was clearly correlated. Our findings indicated that the activation of complement cascade decreases the AQP4 expression in skeletal muscle fibers in inflammatory myopathies, and causes functional defect of skeletal muscle fibers in inflammatory myopathies.

水通道蛋白4(Aquaporin 4，AQP4)不仅在调节多种细胞的水分平衡中起重要作用，而且是视神经脊髓炎的自身免疫靶点。在这个项目中，我们研究了AQP4在包括炎症性肌病在内的各种神经肌肉疾病中的表达。免疫组织化学显示，炎症性肌病中除再生肌纤维和失神经肌纤维外，许多非坏死性肌纤维也表现为AQP4表达降低。在与AQP4表达降低相关的因素分析中，膜攻击复合体(C5b9)在肌肉组织中的沉积与AQP4的表达有明显的相关性。我们的结果表明，在炎症性肌病中，补体激活级联降低了骨骼肌纤维中AQP4的表达，从而导致了炎症性肌病中骨骼肌纤维的功能缺陷。

项目成果

Apoptosis of primary sensory neurons in GD 1b-induced sensoryataxic neuropathy

GD 1b 诱导的感觉神经病中初级感觉神经元的凋亡

• 发表时间: 2008
• 期刊: Exp Neurol 209
• 作者: Kaida K;Kusunoki S;Kanzaki M;Kaida K;Kaida K;Furiya Y;Takada K
• 通讯作者: Takada K

悪性腫瘍関連筋炎

恶性肿瘤相关性肌炎

• 发表时间: 2010
• 期刊: Brain and Nerve
• 作者: Yugeta A;Terao Y;Fukuda H;Hikosaka O;Yokochi F;Okiyama R;Taniguchi M;Takahashi H;Hamada I;Hanajima R;Ugawa Y;清水潤
• 通讯作者: 清水潤

Immunohistochemical and electron microscopy study of myositis with rheumatoid arthritis.

类风湿性关节炎肌炎的免疫组织化学和电子显微镜研究。

• 发表时间: 2010
• 作者: Shimizu J;Hashimoto M;Kadoya M;Koide Y;Kubota A;Tsuji S
• 通讯作者: Tsuji S

膠原病に伴う筋炎の筋病理像の解析

胶原病相关肌炎的肌病理学图像分析

• 发表时间: 2008
• 作者: 清水潤;橋本明子;辻省次
• 通讯作者: 辻省次

IFNβ-1b may severely exacerbate Japanese optic-spinal MS in neuromyelitis optica spectrum

• DOI: 10.1212/wnl.0b013e3181f8832e
• 发表时间: 2010-10-19
• 期刊: NEUROLOGY
• 影响因子: 9.9
• 作者: Shimizu, J.;Hatanaka, Y.;Tsuji, S.
• 通讯作者: Tsuji, S.