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Endoplasmic reticulum stress;Multi-functional organelle endoplasmic reticulum regulates the fates of cells and bodies.The degradation of mutant proteins by ERAD and the pathogenesis of diseases. Osaki Yosuke 1 , Saito Atsushi 2 , Imaizumi Kazunori 3 1Department of Biochemistry, Institute of Biomedical & Health Sciences, Hiroshima University/Department of Nephrology, Hiroshima University Hospital, Hiroshima, Japan. 2Department of Stress Protein Processing, Institute of Biomedical & Health Sciences, Hiroshima University, Hiroshima, Japan. 3Department of Biochemistry, Institute of Biomedical & Health Sciences, Hiroshima University, Hiroshima, Japan. pp.1684-1689
Published Date 2018/11/28
DOI https://doi.org/10.20837/42018121684
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 ER associated degradation(ERAD)is known as a degradation pathway mediated by the ubiquitin-proteasome system. Recent studies have indicated that the degradation of mutant proteins by ERAD is involved in the pathogenesis of various diseases. Mutant proteins caused by gene mutation is degraded by ERAD, resulting in losing their biological functions and development of diseases. On the other hand, the mutant proteins are sorted to appropriate sites by escaping the degradation of ERAD, followed by acquiring the functions. Here, we review the degradation system of mutant proteins and the importance of ERAD in the development of diseases.



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電子版ISSN 印刷版ISSN 0917-5857 医薬ジャーナル社

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