The nucleus is the repository for the eukaryotic cell's genetic blueprint, which must be protected from harm to ensure survival. Multiple quality control (QC) pathways operate in the nucleus to maintain the integrity of the DNA, the fidelity of the DNA code during replication, its transcription into mRNA, and the functional structure of the proteins that are required for DNA maintenance, mRNA transcription, and other important nuclear processes. Although we understand a great deal about DNA and RNA QC mechanisms, we know far less about nuclear protein quality control (PQC) mechanisms despite that fact that many human diseases are causally linked to protein misfolding in the nucleus. In this review, we discuss what is known about nuclear PQC and we highlight new questions that have emerged from recent developments in nuclear PQC studies.

中文翻译：

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蛋白质质量控​​制在细胞核中。

核是真核细胞遗传蓝图的储存库，必须对其进行保护以免受伤害，以确保生存。多种质量控制（QC）途径在细胞核中起作用，以维持DNA的完整性，复制过程中DNA编码的保真度，其转录成mRNA以及DNA维持，mRNA转录所需的蛋白质功能结构，和其他重要的核过程。尽管我们对DNA和RNA QC机制了解很多，但对核蛋白质量控制（PQC）机制的了解却少得多，尽管事实是许多人类疾病与蛋白在核中的错误折叠有因果关系。在这篇综述中，我们讨论了关于核动力品质控制的已知知识，并重点介绍了核动力品质控制研究的最新发展中出现的新问题。