Cited by 1,630Endoplasmic Reticulum Stress and DiseaseHeat shock proteins researchCellular transport and secretion

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

Wang, Miao, Kaufman, Randal J.·2016·Nature

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.

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Crossref · 10.1038/nature17041

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Authors
Wang, M., & Kaufman, R. J.
Affiliations
Sanford Burnham Prebys Medical Discovery Institute (US)
Corresponding author
Kaufman, Randal J.
Venue
Nature
Published
2016-01
Volume
529
Issue
7586
Pages
326-335
ISSN
  • 0028-0836 (ISSN-L)
  • 1476-4687
OpenAlex
W2263498456
Cited by
1,630
References
126
Citations by year
2026: 127 · 2025: 166 · 2024: 177 · 2023: 180 · 2022: 200 · 2021: 179 · 2020: 185 · 2019: 137 · 2018: 120 · 2017: 113 · 2016: 44
FWCI
14.97
Type
journal article
Language
en
Open access
closed
Locations
  • Nature (publishedVersion)
  • PubMed (publishedVersion)
  • RePEc: Research Papers in Economics (submittedVersion)
Funders
  • National Institutes of Health
  • National Cancer Institute (P01CA128814, CA-128814)
  • National Institute of Diabetes and Digestive and Kidney Diseases (R01 DK103183)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Endoplasmic Reticulum Stress and Disease
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Heat shock proteins research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Cellular transport and secretion
Keywords
protein misfolding, endoplasmic reticulum stress, unfolded protein response, secretory pathway, protein folding, protein trafficking, cell fate, human disease, ER-associated degradation
MeSH terms
Animals, Disease, Endoplasmic Reticulum, Humans, Proteins, Protein Folding, Unfolded Protein Response, Molecular Targeted Therapy, Endoplasmic Reticulum Stress

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