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Osmolytes dynamically regulate mutant Huntingtin aggregation and CREB function in Huntington's disease cell models.

Shreyaas Aravindan1, Samantha Chen1, Hannaan Choudhry1

  • 1Department of Cell Biology and Neuroscience, Rutgers State University of New Jersey, Nelson Biology Laboratory, 604 Allison Road, Piscataway, NJ, 08854, USA.

Scientific Reports
|September 24, 2020
PubMed
Summary

Stabilizing osmolytes promote mutant Huntingtin (mHtt) aggregation, alleviating CREB transcription factor dysfunction and improving cell survival in Huntington's disease models. This suggests smaller mHtt species are key in neurodegeneration.

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Area of Science:

  • Biochemistry
  • Neuroscience
  • Cell Biology

Background:

  • Huntington's disease (HD) is linked to mutant Huntingtin (mHtt) aggregation.
  • Intrinsically disordered proteins like mHtt are challenging to study.
  • Osmolytes modulate protein folding and aggregation.

Purpose of the Study:

  • To investigate the role of osmolytes in mHtt aggregation and pathogenicity.
  • To explore the impact of mHtt aggregation on transcription factor function and cell survival.
  • To test the hypothesis that soluble mHtt species drive neurodegeneration.

Main Methods:

  • Utilized inducible PC12 and striatal neuron cell models of HD.
  • Applied stabilizing polyol osmolytes and the destabilizing osmolyte urea.
  • Assessed mHtt aggregation into inclusion bodies (IBs) via microscopy.
  • Measured CREB transcription factor activity and cell survival under stress.
  • Investigated the role of HSP70 and HSC70 chaperones.

Main Results:

  • Stabilizing osmolytes induced mHtt aggregation into IBs, while urea did not.
  • Osmolyte-induced IB formation correlated with improved cell survival under stress.
  • Diffuse mHtt repressed CREB activity, which was mitigated by osmolytes or urea.
  • HSP70/HSC70 knockdown did not affect osmolyte-induced aggregation.

Conclusions:

  • Stabilizing polyol osmolytes promote mHtt aggregation, alleviating CREB dysfunction and enhancing cell survival.
  • Osmolytes offer a novel approach to modulate mHtt aggregation and toxicity.
  • Findings support the hypothesis that soluble, lower molecular weight mHtt species are pathogenic in HD.