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8P34

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

Summary for 8P34
Entry DOI10.2210/pdb8p34/pdb
EMDB information17383
DescriptorMicrotubule-associated protein tau (1 entity in total)
Functional Keywordsdeltak281, tau, amyloid filament, cross-beta-sheet, protein fibril
Biological sourceHomo sapiens (human)
Total number of polymer chains1
Total formula weight10135.67
Authors
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. (deposition date: 2023-05-17, release date: 2023-07-05, Last modification date: 2024-07-24)
Primary citationSchweighauser, 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
Cited by
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
PDB entries with the same primary citation
Experimental method
ELECTRON MICROSCOPY (2.61 Å)
Structure validation

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