9ERN

CTE type II tau filament from vacuolar tauopathy

9ERN の概要

• エントリーDOI: 10.2210/pdb9ern/pdb
• 関連するPDBエントリー: 9ERM
• EMDBエントリー: 19927
• 分子名称: Microtubule-associated protein tau (1 entity in total)
• 機能のキーワード: tau filament, cte type ii, vacuolar tauopathy, protein fibril
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 6
• 化学式量合計: 275519.23

構造登録者

Qi, C.,Scheres, H.W.S.,Goedert, M. (登録日: 2024-03-24, 公開日: 2024-05-29)

主引用文献

Qi, C.,Kobayashi, R.,Kawakatsu, S.,Kametani, F.,Scheres, S.H.W.,Goedert, M.,Hasegawa, M.
Tau filaments with the chronic traumatic encephalopathy fold in a case of vacuolar tauopathy with VCP mutation D395G.
Acta Neuropathol, 147:86-86, 2024

PubMed Abstract: Dominantly inherited mutation D395G in the gene encoding valosin-containing protein causes vacuolar tauopathy, a type of behavioural-variant frontotemporal dementia, with marked vacuolation and abundant filamentous tau inclusions made of all six brain isoforms. Here we report that tau inclusions were concentrated in layers II/III of the frontotemporal cortex in a case of vacuolar tauopathy. By electron cryomicroscopy, tau filaments had the chronic traumatic encephalopathy (CTE) fold. Tau inclusions of vacuolar tauopathy share this cortical location and the tau fold with CTE, subacute sclerosing panencephalitis and amyotrophic lateral sclerosis/parkinsonism-dementia complex, which are believed to be environmentally induced. Vacuolar tauopathy is the first inherited disease with the CTE tau fold.

PubMed: 38758288
DOI: 10.1007/s00401-024-02741-x

実験手法

ELECTRON MICROSCOPY (2.5 Å)