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

Author

Listed:
  • Miao Wang

    (Degenerative Diseases Program, Sanford Burnham Prebys Medical Discovery Institute)

  • Randal J. Kaufman

    (Degenerative Diseases Program, Sanford Burnham Prebys Medical Discovery Institute)

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.

Suggested Citation

  • Miao Wang & Randal J. Kaufman, 2016. "Protein misfolding in the endoplasmic reticulum as a conduit to human disease," Nature, Nature, vol. 529(7586), pages 326-335, January.
    • Handle: RePEc:nat:nature:v:529:y:2016:i:7586:d:10.1038_nature17041
    DOI: 10.1038/nature17041
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    Cited by:

    1. Yu-Jie Chen & Jeffrey Knupp & Anoop Arunagiri & Leena Haataja & Peter Arvan & Billy Tsai, 2021. "PGRMC1 acts as a size-selective cargo receptor to drive ER-phagic clearance of mutant prohormones," Nature Communications, Nature, vol. 12(1), pages 1-17, December.
    2. Iqbal Dulloo & Peace Atakpa-Adaji & Yi-Chun Yeh & Clémence Levet & Sonia Muliyil & Fangfang Lu & Colin W. Taylor & Matthew Freeman, 2022. "iRhom pseudoproteases regulate ER stress-induced cell death through IP3 receptors and BCL-2," Nature Communications, Nature, vol. 13(1), pages 1-18, December.
    3. Chaiheon Lee & Mingyu Park & W. C. Bhashini Wijesinghe & Seungjin Na & Chae Gyu Lee & Eunhye Hwang & Gwangsu Yoon & Jeong Kyeong Lee & Deok-Ho Roh & Yoon Hee Kwon & Jihyeon Yang & Sebastian A. Hughes , 2024. "Oxidative photocatalysis on membranes triggers non-canonical pyroptosis," Nature Communications, Nature, vol. 15(1), pages 1-16, December.
    4. Eun-Ji Park & Hyun-Soo Kim & Do-Hyoung Lee & Su-Min Kim & Joon-Sup Yoon & Ji-Min Lee & Se Jin Im & Ho Lee & Min-Woo Lee & Chang-Woo Lee, 2023. "Ssu72 phosphatase is essential for thermogenic adaptation by regulating cytosolic translation," Nature Communications, Nature, vol. 14(1), pages 1-17, December.
    5. Li Qi jun, 2018. "TG could Modulate FPN1 in MES 23.5 Cells by Hepcidin," International Journal of Sciences, Office ijSciences, vol. 7(09), pages 52-55, September.

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