The endoplasmic reticulum is a key site for protein production and quality control. More than one-third of proteins are synthesized and folded into the correct three-dimensional conformation in the endoplasmic reticulum. However, during protein folding, unfolded and/or misfolded proteins are prone to occur, which may lead to endoplasmic reticulum stress. Organisms can monitor the quality of the proteins produced by endoplasmic reticulum quality control (ERQC) and endoplasmic reticulum-associated degradation (ERAD), which maintain endoplasmic reticulum protein homeostasis by degrading abnormally folded proteins. The underlying mechanisms of protein folding and ERAD in mammals have not yet been fully explored. Therefore, this paper reviews the process and function of protein folding and ERAD in mammalian cells, in order to help clinicians better understand the mechanism of ERAD and to provide a scientific reference for the treatment of diseases caused by abnormal ERAD.
内质网是体内蛋白质产生和质量控制的关键场所，体内约1/3的蛋白是经内质网合成并加工发挥作用。蛋白质合成后需要进行一定的折叠，形成正确的三维构象，从而发挥其功能。然而在蛋白质折叠过程中易出现错误，导致未折叠和（或）错误折叠蛋白在内质网内聚集，进而导致内质网应激。对此，机体可通过内质网质量控制（ERQC）和内质网相关降解（ERAD）对生成的蛋白质质量进行检测，并通过降解异常折叠蛋白质，以维持内质网内蛋白稳态。目前为止，对哺乳动物体内有关蛋白质折叠和ERAD的详细机制描述尚不全面。为此，本文综述了哺乳动物细胞中蛋白质折叠与ERAD的过程和功能，并重点对ERAD相关蛋白质构象疾病的潜在病理生理学进行了介绍。.
内质网是体内蛋白质产生和质量控制的关键场所，体内约1/3的蛋白是经内质网合成并加工发挥作用。蛋白质合成后需要进行一定的折叠，形成正确的三维构象，从而发挥其功能。然而在蛋白质折叠过程中易出现错误，导致未折叠和（或）错误折叠蛋白在内质网内聚集，进而导致内质网应激。对此，机体可通过内质网质量控制（ERQC）和内质网相关降解（ERAD）对生成的蛋白质质量进行检测，并通过降解异常折叠蛋白质，以维持内质网内蛋白稳态。目前为止，对哺乳动物体内有关蛋白质折叠和ERAD的详细机制描述尚不全面。为此，本文综述了哺乳动物细胞中蛋白质折叠与ERAD的过程和功能，并重点对ERAD相关蛋白质构象疾病的潜在病理生理学进行了介绍。
Keywords: Endoplasmic reticulum-associated degradation (ERAD); Protein folding; Retrotranslocation; Ubiquitination.