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Neurodegenerative disease

The stress of misfolding

Nature Reviews Neuroscience volume 13, pages 290–291 (2012)Cite this article

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An Erratum to this article was published on 18 April 2012

This article has been updated

The accumulation of unfolded or misfolded proteins in cells causes ER stress that can induce the unfolded protein response (UPR). The UPR aims to protect against the toxic accumulation of such proteins, partly through the elevation of chaperone protein expression. Previous in vitro studies have shown that high levels of α-synuclein can induce ER stress.

The two new studies used the A53TαSTg mouse model of α-synucleinopathy, in which mice express a human-disease-linked variant of α-synuclein, to reveal that mutant α-synuclein expression is also associated with ER stress in vivo. The authors showed that, in these mice, the expression of various UPR-linked chaperone proteins increased with disease progression in the spinal cord and the brain stem — two CNS regions that showed notable α-synuclein-linked pathology.

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  • 18 April 2012

    On page 291 of this highlight, the titles of the original papers were incorrectly given. The correct titles are 'Endoplasmic reticulum stress is important for the manifestations of α-synucleinopathy in vivo' and 'Accumulation of toxic α-synuclein oligomer within endoplasmic reticulum occurs in α-synucleinopathy in vivo'. These have been corrected in the online version.

ORIGINAL RESEARCH PAPERS

  1. Colla, E. et al. Endoplasmic reticulum stress is important for the manifestations of α-synucleinopathy in vivo. J. Neurosci. 32, 3306–3320 (2012)Article

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Colla, E. et al. Accumulation of toxic α-synuclein oligomer within endoplasmic reticulum occurs in α-synucleinopathy in vivo. J. Neurosci. 32, 3301–3305 (2012)Article

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Authors
  1. Darran Yates
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Yates, D. The stress of misfolding. Nat Rev Neurosci 13, 290–291 (2012). https://doi.org/10.1038/nrn3235

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