Tau Isoform Profile in Essential Tremor Diverges From Other Tauopathies

Soong Ho Kim, Kurt Farrell, Stephanie Cosentino, Jean Paul G. Vonsattel, Phyllis L. Faust, Etty P. Cortes, David A. Bennet, Elan D. Louis, John F. Crary

Research output: Contribution to journalArticlepeer-review

Abstract

Patients with essential tremor (ET) frequently develop concurrent dementia, which is often assumed to represent co-morbid Alzheimer disease (AD). Autopsy studies have identified a spectrum of tau pathologies in ET and tau isoforms have not been examined in ET. We performed immunoblotting using autopsy cerebral cortical tissue from patients with ET (n = 13), progressive supranuclear palsy ([PSP], n = 10), Pick disease ([PiD], n = 2), and AD (n = 7). Total tau in ET samples was similar to that in PSP and PiD but was significantly lower than that in AD. Abnormal tau levels measured using the AT8 phospho-tau specific (S202/T205/S208) monoclonal antibody in ET were similar to those in PSP but were lower than in PiD and AD. In aggregates, tau with 3 microtubule-binding domain repeats (3R) was significantly higher in AD than ET, while tau with 4 repeats (4R) was significantly higher in PSP. Strikingly, the total tau without N-terminal inserts in ET was significantly lower than in PSP, PiD, and AD, but total tau with other N-terminal inserts was not. Monomeric tau with one insert in ET was similar to that in PSP and PiD was lower than in AD. Thus, ET brains exhibit an expression profile of tau protein isoforms that diverges from that of other tauopathies.

Original languageEnglish (US)
Pages (from-to)835-843
Number of pages9
JournalJournal of neuropathology and experimental neurology
Volume80
Issue number9
DOIs
StatePublished - Sep 27 2021

Keywords

  • Alzheimer disease
  • Essential tremor
  • Neurodegeneration
  • Neurofibrillary tangle
  • Pick disease
  • Progressive supranuclear palsy
  • Tau isoform
  • Tauopathy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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