臨床神経学

第49回日本神経学会総会

<シンポジウム2-1>神経変性疾患研究の焦点―新たな病的因子の登場と臨床への展望―
基調講演:神経変性疾患研究の課題

高橋 良輔

京都大学大学院医学研究科臨床神経学〔〒606-8507 京都市左京区聖護院川原町54〕

Although the pathogenetic mechanisms underlying neurodegenerative diseases have been long elusive, recent progress in molecular neurogenetics and neurobiology has suggested that accumulation of misfolded protein leads to dysfunction and degeneration of neurons. Misfolded proteins have propensities to form fibrils termed amyloid fibrils. In the process of amyloid fibrils, intermediate forms such as oligomers and protofibrils are produced and thought to have cytotoxic effects to neurons. Neurotoxicity mediated by misfolded proteins are also caused by stress response such as unfolded protein response. Moreover, recent findings indicate that non-neuronal cells surrounding neurons or extracellular misfolded proteins promote neurodegeneration. To eliminate toxic proteins would constitute promising future therapy for neurodegenerative disorders.
Full Text of this Article in Japanese PDF (389K)

(臨床神経, 48:903−905, 2008)
key words:コンフォメーション病, ミスフォールドタンパク質, オリゴマー, 小胞体ストレス, 非細胞自律性

(受付日:2008年5月16日)