Dysregulated ribosome quality control in human diseases.

Tom McGirr, Okan Onar, Seyed Mehdi Jafarnejad
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Abstract

Precise regulation of mRNA translation is of fundamental importance for maintaining homeostasis. Conversely, dysregulated general or transcript-specific translation, as well as abnormal translation events, have been linked to a multitude of diseases. However, driven by the misconception that the transient nature of mRNAs renders their abnormalities inconsequential, the importance of mechanisms that monitor the quality and fidelity of the translation process has been largely overlooked. In recent years, there has been a dramatic shift in this paradigm, evidenced by several seminal discoveries on the role of a key mechanism in monitoring the quality of mRNA translation - namely, Ribosome Quality Control (RQC) - in the maintenance of homeostasis and the prevention of diseases. Here, we will review recent advances in the field and emphasize the biological significance of the RQC mechanism, particularly its implications in human diseases.

人类疾病中的核糖体质量控制失调。
精确调控 mRNA 翻译对维持体内平衡至关重要。相反,一般或转录本特异性翻译失调以及异常翻译事件与多种疾病有关。然而,由于人们误认为 mRNA 的瞬时性使其异常变得无关紧要,因此在很大程度上忽视了监控翻译过程的质量和保真度的机制的重要性。近年来,这种模式发生了巨大转变,一些关于监控 mRNA 翻译质量的关键机制--即核糖体质量控制(RQC)--在维持体内平衡和预防疾病方面的作用的开创性发现就是证明。在此,我们将回顾该领域的最新进展,并强调 RQC 机制的生物学意义,尤其是它对人类疾病的影响。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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