Quality control ensures fidelity in ribosome assembly and cellular health

被引:11
|
作者
Parker, Melissa D. [1 ,2 ]
Karbstein, Katrin [1 ,2 ,3 ]
机构
[1] Scripps Res Inst, Skaggs Grad Sch Chem & Biol Sci, La Jolla, CA 92037 USA
[2] Univ Florida, Scripps Biomed Res, Jupiter, FL 33458 USA
[3] Howard Hughes Med Inst, Chevy Chase, MD 20815 USA
来源
JOURNAL OF CELL BIOLOGY | 2023年 / 222卷 / 04期
关键词
BOWEN-CONRADI SYNDROME; 90S PRE-RIBOSOME; SHWACHMAN-DIAMOND SYNDROME; MESSENGER-RNA TRANSLATION; SUBUNIT PRECURSORS; CYTOPLASMIC MATURATION; FUNCTIONAL-ANALYSIS; MUTATIONAL ANALYSIS; INITIATION-FACTOR; BLACKFAN ANEMIA;
D O I
10.1083/jcb.202209115
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Melissa Parker and Katrin Karbstein review our current understanding of the quality control mechanisms governing ribosome assembly. The coordinated integration of ribosomal RNA and protein into two functional ribosomal subunits is safeguarded by quality control checkpoints that ensure ribosomes are correctly assembled and functional before they engage in translation. Quality control is critical in maintaining the integrity of ribosomes and necessary to support healthy cell growth and prevent diseases associated with mistakes in ribosome assembly. Its importance is demonstrated by the finding that bypassing quality control leads to misassembled, malfunctioning ribosomes with altered translation fidelity, which change gene expression and disrupt protein homeostasis. In this review, we outline our understanding of quality control within ribosome synthesis and how failure to enforce quality control contributes to human disease. We first provide a definition of quality control to guide our investigation, briefly present the main assembly steps, and then examine stages of assembly that test ribosome function, establish a pass-fail system to evaluate these functions, and contribute to altered ribosome performance when bypassed, and are thus considered "quality control."
引用
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页数:20
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