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論文
タイトル
タイトル(英)
Ultrastructures of α-synuclein filaments in synucleinopathy brains and experimental models.
参照URL
https://researchmap.jp/masatohasegawa/published_papers/44097750
著者
著者(英)
Airi Tarutani,Masato Hasegawa
担当区分
概要
概要(英)
Intracellular α-synuclein (α-syn) inclusions are a neuropathological hallmark of Lewy body disease (LBD) and multiple system atrophy (MSA), both of which are termed synucleinopathy. LBD is defined by Lewy bodies and Lewy neurites in neurons, while MSA displays glial cytoplasmic inclusions in oligodendrocytes. Pathological α-syn adopts an ordered filamentous structure with a 5-10 nm filament diameter, and this conformational change has been suggested to be involved in the disease onset and progression. Synucleinopathy also exhibits characteristic ultrastructural and biochemical properties of α-syn filaments, and α-syn strains with distinct conformations have been identified. Numerous experimental studies have supported the idea that pathological α-syn self-amplifies and spreads throughout the brain, during which processes the conformation of α-syn filaments may drive the disease specificity. In this review, we summarize the ultrastructural features and heterogeneity of α-syn filaments in the brains of patients with synucleinopathy and in experimental models of seeded α-syn aggregation.
出版者・発行元
出版者・発行元(英)
誌名
誌名(英)
Journal of movement disorders
開始ページ
終了ページ
出版年月
2023年11月22日
査読の有無
招待の有無
掲載種別
研究論文(学術雑誌)
ISSN
DOI URL
https://doi.org/10.14802/jmd.23213
共同研究・競争的資金等の研究課題
研究者