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Nature. 2016 Jan 21;529(7586):326-35. doi: 10.1038/nature17041.

Protein misfolding in the endoplasmic reticulum as a conduit to human disease.

Author information

1
Degenerative Diseases Program, Sanford Burnham Prebys Medical Discovery Institute, 10901 North Torrey Pines Road, La Jolla, California 92037, USA.

Abstract

In eukaryotic cells, the endoplasmic reticulum is essential for the folding and trafficking of proteins that enter the secretory pathway. Environmental insults or increased protein synthesis often lead to protein misfolding in the organelle, the accumulation of misfolded or unfolded proteins - known as endoplasmic reticulum stress - and the activation of the adaptive unfolded protein response to restore homeostasis. If protein misfolding is not resolved, cells die. Endoplasmic reticulum stress and activation of the unfolded protein response help to determine cell fate and function. Furthermore, endoplasmic reticulum stress contributes to the aetiology of many human diseases.

PMID:
26791723
DOI:
10.1038/nature17041
[Indexed for MEDLINE]

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