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.

中文翻译：

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应对蛋白质质量控​​制失败

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严酷的条件。蛋白质对于执行绝大多数细胞和机体功能至关重要。为了维持健康的蛋白质组，细胞依赖于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，而且对于监视，预防和挽救蛋白质也是必不可少的蛋白质缺陷。PQC系统的主要参与者是伴侣蛋白和蛋白质降解系统：泛素-蛋白酶体系统和自噬。在这里，我们提供了健康和疾病真核细胞中各种PQC系统的综合概述，重点是关键的监管方面及其相互影响。