Pridopidine reduces mutant huntingtin‐induced endoplasmic reticulum stress by modulation of the Sigma‐1 receptor

@article{Shenkman2021PridopidineRM,
  title={Pridopidine reduces mutant huntingtin‐induced endoplasmic reticulum stress by modulation of the Sigma‐1 receptor},
  author={Marina Shenkman and Michael Geva and Noga Gershoni-Emek and Michael R. Hayden and Gerardo Z. Lederkremer},
  journal={Journal of Neurochemistry},
  year={2021},
  volume={158},
  pages={467 - 481}
}
The endoplasmic reticulum (ER)‐localized Sigma‐1 receptor (S1R) is neuroprotective in models of neurodegenerative diseases, among them Huntington disease (HD). Recent clinical trials in HD patients and preclinical studies in cellular and mouse HD models suggest a therapeutic potential for the high‐affinity S1R agonist pridopidine. However, the molecular mechanisms of the cytoprotective effect are unclear. We have previously reported strong induction of ER stress by toxic mutant huntingtin (mHtt… 
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