Association between axonal damage and molecular basis in demyelinating neuropathies: prognosis and response to treatment

轴突损伤与脱髓鞘神经病分子基础之间的关联：预后和治疗反应

• 批准号: 22590928
• 负责人: KAWAGASHIRA Yuichi
• 金额: $ 2.91万
• 依托单位: Nagoya University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2010
• 资助国家: 日本
• 起止时间: 2010 至 2012
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-22590928/
• 关键词: 脱髄性ニューロパチー; 軸索障害; Axon-myelin interaction; 治療反応性; イオンチャネル; 脱髄; 末梢神経; チャネル; axon-glial interaction; TAG-1; CIDP; 慢性炎症性脱髄性多発ニューロパチー(CIDP); Intravenous immunoglobulin(IVIg); 軸索機能障害; 一塩基多型(single nucleotide polymorphism,SNP)

The association between axon-myelin interaction and functional several molecules which are distributed from node of Ranvie to juxtaparanode is well recognized. In this study, we analyzed immunohistochemically the distribution and expression of those molecules, including sodium channel, TAG-1, CNTN1, Caspr and Caspr2, using by teased-fiber preparation in patients with demyelinating neuropathies. The expression of sodium channel in demyelinating neuropathies showed markedly decrease compared with normal control although no morphological changes are observed in light microscopic study. In addition, we confirmed that homologous TAG-1 knockout mice resulted in significant ateliosis. These results may suggest that several molecules which is associated with axon-myelin interaction, including TAG-1, express their function in the development phase and play an important role in repair after demyelination.

轴突-髓鞘相互作用与从Ranvie结到Anastaparanode的几种功能分子之间的关联是公认的。本研究采用酶联免疫吸附法（ELISA）检测脱髓鞘性神经病患者神经组织中钠通道、TAG-1、CNTN 1、Caspr和Caspr 2等分子的分布和表达。光镜下无形态学改变，但脱髓鞘神经病组钠通道表达明显减少。此外，我们证实同源TAG-1敲除小鼠导致显著的发育不全。这些结果可能表明，与轴突-髓鞘相互作用相关的几种分子，包括TAG-1，在发育阶段表达其功能，并在脱髓鞘后的修复中发挥重要作用。

项目成果

Characteristics of hypertrophic form in chronic inflammatory demyelinating polyneuropathy. Inflammatory Neuropathy consortium

慢性炎症性脱髓鞘性多发性神经病肥厚型的特征。

• 发表时间: 2012
• 作者: Masahiro Iijima;Ken Ohyama;Rina Hashimoto;Minoru Tomita;Yuichi Kawagashira;Haruki Koike;Gen Sobue.
• 通讯作者: Gen Sobue.

Pyramidal tract involvement in Guillain-Barrテゥ syndrome associated with anti-GM1 antibody

与抗 GM1 抗体相关的吉兰-巴尔综合征的锥体束受累

• 发表时间: 2010
• 期刊: J Neurol Neurosurg Psychiatry.
• 作者: Tomita M;et. al.
• 通讯作者: et. al.

抗Myelin-associated-glycoprotein抗体陽性IgM-MGUSニューロパチーにおけるRituximab療法の有用性

利妥昔单抗治疗在抗髓磷脂相关糖蛋白抗体阳性 IgM-MGUS 神经病中的作用

• 发表时间: 2012
• 作者: Tomita M;et. al.;川頭祐一(名古屋大学 神経内科),大山健,橋本里奈,冨田稔,飯島正博,小池春樹,祖父江元
• 通讯作者: 川頭祐一(名古屋大学 神経内科),大山健,橋本里奈,冨田稔,飯島正博,小池春樹,祖父江元

Systemic but asymptomatic transthyretin amyloidosis 8 years after domino liver transplantation

• DOI: 10.1136/jnnp.2010.218958
• 发表时间: 2011-11-01
• 期刊: JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
• 影响因子: 11
• 作者: Koike, Haruki;Kiuchi, Tetsuya;Sobue, Gen
• 通讯作者: Sobue, Gen

IgM MGUS ANTI-MAG NEUROPATHY WITH PREDOMINANT MUSCLE WEAKNESS AND EXTENSIVE MUSCLE ATROPHY

• DOI: 10.1002/mus.21741
• 发表时间: 2010-09-01
• 期刊: MUSCLE & NERVE
• 影响因子: 3.4
• 作者: Kawagashira, Yuichi;Kondo, Naohide;Sobue, Gen
• 通讯作者: Sobue, Gen