Item request has been placed! ×
Item request cannot be made. ×
loading  Processing Request

Tau filaments from amyotrophic lateral sclerosis/parkinsonism-dementia complex adopt the CTE fold

Item request has been placed! ×
Item request cannot be made. ×
loading   Processing Request
Read Online Read More Add to Saved list
  • Additional Information
    • Publisher Information:
      Proceedings of the National Academy of Sciences http://dx.doi.org/10.1073/pnas.2306767120 Proceedings of the National Academy of Sciences 2023-12-20T15:25:12Z 2023-12-19 2023-04-26 2023-12-20T15:25:11Z
    • Abstract:
      Peer reviewed: True
      Publication status: Published
      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.
    • Subject Terms:
      3101 Biochemistry and Cell Biology; 31 Biological Sciences; Frontotemporal Dementia (FTD); Brain Disorders; ALS; Acquired Cognitive Impairment; Dementia; Rare Diseases; Neurosciences; Neurodegenerative; 2 Aetiology; 2.1 Biological and endogenous factors; Neurological; Article
    • Availability:
      Open access content. Open access content
    • Note:
      application/zip
      text/xml
      application/pdf
      English
    • Other Numbers:
      HS1 oai:www.repository.cam.ac.uk:1810/362543
      1488667071
    • Contributing Source:
      UNIV OF CAMBRIDGE
      From OAIster®, provided by the OCLC Cooperative.
    • Accession Number:
      edsoai.on1488667071
HoldingsOnline