8OTD

TMEM106B Fold1-s filament from Guam ALS/PDC

8OTD の概要

• エントリーDOI: 10.2210/pdb8otd/pdb
• 関連するPDBエントリー: 8OT9
• EMDBエントリー: 17175
• 分子名称: Transmembrane protein 106B (1 entity in total)
• 機能のキーワード: tmem106b fold1-single filaments, guam als/pdc, protein fibril
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 4
• 化学式量合計: 124569.18

構造登録者

Qi, C.,Yang, S.,Scheres, S.H.W.,Goedert, M. (登録日: 2023-04-20, 公開日: 2024-03-27, 最終更新日: 2024-10-09)

主引用文献

Qi, C.,Verheijen, B.M.,Kokubo, Y.,Shi, Y.,Tetter, S.,Murzin, A.G.,Nakahara, A.,Morimoto, S.,Vermulst, M.,Sasaki, R.,Aronica, E.,Hirokawa, Y.,Oyanagi, K.,Kakita, A.,Ryskeldi-Falcon, B.,Yoshida, M.,Hasegawa, M.,Scheres, S.H.W.,Goedert, M.
Tau filaments from amyotrophic lateral sclerosis/parkinsonism-dementia complex adopt the CTE fold.
Proc.Natl.Acad.Sci.USA, 120:e2306767120-e2306767120, 2023

PubMed Abstract: The amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) of the island of Guam and the Kii peninsula of Japan is a fatal neurodegenerative disease of unknown cause that is characterized by the presence of abundant filamentous tau inclusions in brains and spinal cords. Here, we used electron cryo-microscopy to determine the structures of tau filaments from the cerebral cortex of three cases of ALS/PDC from Guam and eight cases from Kii, as well as from the spinal cord of two of the Guam cases. Tau filaments had the chronic traumatic encephalopathy (CTE) fold, with variable amounts of Type I and Type II filaments. Paired helical tau filaments were also found in three Kii cases and tau filaments with the corticobasal degeneration fold in one Kii case. We identified a new Type III CTE tau filament, where protofilaments pack against each other in an antiparallel fashion. ALS/PDC is the third known tauopathy with CTE-type filaments and abundant tau inclusions in cortical layers II/III, the others being CTE and subacute sclerosing panencephalitis. Because these tauopathies are believed to have environmental causes, our findings support the hypothesis that ALS/PDC is caused by exogenous factors.

PubMed: 38100415
DOI: 10.1073/pnas.2306767120

実験手法

ELECTRON MICROSCOPY (2.6 Å)