Neurodegenerative Disorders with Tauopathies: Mad Tau Diseases?

  • L. Buée
  • C. Mailliot
  • T. Bussière
  • N. Sergeant
  • V. Buée-Scherrer
  • P. R. Hof
  • S. Flament
  • A. Delacourte
Conference paper
Part of the Research and Perspectives in Alzheimer’s Disease book series (ALZHEIMER)

Abstract

In neurodegenerative disorders, referred to as tauopathies, including Alzheimer’s disease, hyperphosphorylated tau proteins are found aggregated into filaments. Comparative biochemistry of tau aggregates shows that they differ in both phosphorylation and content of tau isoforms. Six tau isoforms are found in human brain that contain either three (3R) or four microtubule-binding domains (4R). In Alzheimer’s disease, all six tau isoforms are abnormally phosphorylated and aggregate into paired helical filaments. They are detected by immunoblotting as a major tau triplet (tau55, 64 and 69). Similar properties are found in amyotrophic lateral sclerosis parkinsonism-dementia complex of Guam, Down’s syndrome, some forms of hereditary fronto-temporal dementia and postencephalitic parkinsonism. In corticobasal degeneration and progressive supranuclear palsy, only 4R-tau isoforms aggregate into twisted and straight filaments, respectively. They appear as a major tau doublet (tau64 and 69). Some forms of hereditary fronto-temporal dementia also display these tau features. Finally, in Pick’s disease, only 3R-tau isoforms aggregate into random coiled filaments. They are characterized by another major tau doublet (tau55 and 64). These differences in tau isoforms may be related to 1) mutations/polymorphisms on tau gene, 2) the degeneration of particular cell populations in a given disorder or 3) aberrant cell trafficking of particular tau isoforms.

Keywords

Progressive Supranuclear Palsy Progressive Supranuclear Palsy Corticobasal Degeneration Paired Helical Filament Cassette Exon 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • L. Buée
  • C. Mailliot
  • T. Bussière
  • N. Sergeant
  • V. Buée-Scherrer
  • P. R. Hof
  • S. Flament
  • A. Delacourte

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