MRC Laboratory of Molecular Biology, Cambridge CB2 0QH, United Kingdom; email:
Annu Rev Cell Dev Biol. 2017 Oct 6;33:439-465. doi: 10.1146/annurev-cellbio-111315-125334.
Cells and organisms have evolved numerous mechanisms to cope with and to adapt to unexpected challenges and harsh conditions. Proteins are essential to perform the vast majority of cellular and organismal functions. To maintain a healthy proteome, cells rely on a network of factors and pathways collectively known as protein quality control (PQC) systems, which not only ensure that newly synthesized proteins reach a functional conformation but also are essential for surveillance, prevention, and rescue of protein defects. The main players of PQC systems are chaperones and protein degradation systems: the ubiquitin-proteasome system and autophagy. Here we provide an integrated overview of the diverse PQC systems in eukaryotic cells in health and diseases, with an emphasis on the key regulatory aspects and their cross talks. We also highlight how PQC regulation may be exploited for potential therapeutic benefit.
细胞和生物体已经进化出许多机制来应对和适应意外的挑战和恶劣的条件。蛋白质对于执行绝大多数细胞和生物体功能至关重要。为了维持健康的蛋白质组，细胞依赖于一个被称为蛋白质质量控制（PQC）系统的因素和途径网络，该系统不仅确保新合成的蛋白质达到功能构象，而且对于蛋白质缺陷的监测、预防和挽救也是必不可少的。PQC 系统的主要参与者是伴侣蛋白和蛋白降解系统：泛素-蛋白酶体系统和自噬。在这里，我们提供了一个健康和疾病状态下真核细胞中不同 PQC 系统的综合概述，重点介绍了关键的调节方面及其相互作用。我们还强调了 PQC 调节如何可以被利用来获得潜在的治疗益处。
