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特集 プロテイノパチーの神経病理学
文献概要
はじめに
TDP-43は前頭側頭葉変性症（frontotemporal lobar degeneration：FTLD）と筋萎縮性側索硬化症（amyotrophic lateral sclerosis：ALS）を病理学的に特徴づける分子である。本稿ではまずFTLDという，ややわかりにくい疾患群の歴史的背景を説明し，次いでTDP-43異常蓄積の発見とその後の病理学的研究の展開について述べる。後半では，さまざまな遺伝子や蛋白の異常，実験室モデルなどからみたTDP-43プロテイノパチーの病態解析の現状について解説を試みる。FTLDやALSにおけるTDP-43異常蓄積の発見は，神経変性疾患に関わる新たな研究領域の出現をもたらすことになった。
TDP-43は前頭側頭葉変性症（frontotemporal lobar degeneration：FTLD）と筋萎縮性側索硬化症（amyotrophic lateral sclerosis：ALS）を病理学的に特徴づける分子である。本稿ではまずFTLDという，ややわかりにくい疾患群の歴史的背景を説明し，次いでTDP-43異常蓄積の発見とその後の病理学的研究の展開について述べる。後半では，さまざまな遺伝子や蛋白の異常，実験室モデルなどからみたTDP-43プロテイノパチーの病態解析の現状について解説を試みる。FTLDやALSにおけるTDP-43異常蓄積の発見は，神経変性疾患に関わる新たな研究領域の出現をもたらすことになった。
参考文献
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