4.6 Article

Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3

Journal

CELLS
Volume 11, Issue 19, Pages -

Publisher

MDPI
DOI: 10.3390/cells11193132

Keywords

polyglutamine; ataxin-3; sleep; EEG; beta-oscillations

Categories

Funding

  1. NIH [T32 HL110952, R35NS122302]
  2. George & Lucile Heeringa funds for ataxia research
  3. National Ataxia Foundation (SCA3 Translational Research Award 2019/2020)
  4. University of Michigan
  5. Taubman Emerging Scholars Award at the University of Michigan

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This study quantitatively characterized sleep electroencephalography (EEG) in SCA3 transgenic mice and found that sleep architecture and EEG spectral power were dysregulated, indicating a potential shared etiology between mouse and human SCA3 phenotypes.
Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder showing progressive neuronal loss in several brain areas and a broad spectrum of motor and non-motor symptoms, including ataxia and altered sleep. While sleep disturbances are known to play pathophysiologic roles in other neurodegenerative disorders, their impact on SCA3 is unknown. Using spectrographic measurements, we sought to quantitatively characterize sleep electroencephalography (EEG) in SCA3 transgenic mice with confirmed disease phenotype. We first measured motor phenotypes in 18-31-week-old homozygous SCA3 YACMJD84.2 mice and non-transgenic wild-type littermate mice during lights-on and lights-off periods. We next implanted electrodes to obtain 12-h (zeitgeber time 0-12) EEG recordings for three consecutive days when the mice were 26-36 weeks old. EEG-based spectroscopy showed that compared to wild-type littermates, SCA3 homozygous mice display: (i) increased duration of rapid-eye movement sleep (REM) and fragmentation in all sleep and wake states; (ii) higher beta power oscillations during REM and non-REM (NREM); and (iii) additional spectral power band alterations during REM and wake. Our data show that sleep architecture and EEG spectral power are dysregulated in homozygous SCA3 mice, indicating that common sleep-related etiologic factors may underlie mouse and human SCA3 phenotypes.

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