8P34

Tau filaments extracted from human brain with the DeltaK281 mutation in MAPT

8P34 の概要

• エントリーDOI: 10.2210/pdb8p34/pdb
• EMDBエントリー: 17383
• 分子名称: Microtubule-associated protein tau (1 entity in total)
• 機能のキーワード: deltak281, tau, amyloid filament, cross-beta-sheet, protein fibril
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 10135.67

構造登録者

Schweighauser, M.,Garringer, H.J.,Klingstedt, T.,Masuda-Suzukake, M.,Murrell, J.R.,Vidal, R.,Scheres, S.H.W.,Goedert, M.,Ghetti, B.,Newell, K.L. (登録日: 2023-05-17, 公開日: 2023-07-05, 最終更新日: 2024-07-24)

主引用文献

Schweighauser, M.,Garringer, H.J.,Klingstedt, T.,Nilsson, K.P.R.,Masuda-Suzukake, M.,Murrell, J.R.,Risacher, S.L.,Vidal, R.,Scheres, S.H.W.,Goedert, M.,Ghetti, B.,Newell, K.L.
Mutation ∆K281 in MAPT causes Pick's disease.
Acta Neuropathol, 146:211-226, 2023

PubMed Abstract: Two siblings with deletion mutation ∆K281 in MAPT developed frontotemporal dementia. At autopsy, numerous inclusions of hyperphosphorylated 3R Tau were present in neurons and glial cells of neocortex and some subcortical regions, including hippocampus, caudate/putamen and globus pallidus. The inclusions were argyrophilic with Bodian silver, but not with Gallyas-Braak silver. They were not labelled by an antibody specific for tau phosphorylated at S262 and/or S356. The inclusions were stained by luminescent conjugated oligothiophene HS-84, but not by bTVBT4. Electron cryo-microscopy revealed that the core of tau filaments was made of residues K254-F378 of 3R Tau and was indistinguishable from that of Pick's disease. We conclude that MAPT mutation ∆K281 causes Pick's disease.

PubMed: 37351604
DOI: 10.1007/s00401-023-02598-6

実験手法

ELECTRON MICROSCOPY (2.61 Å)