Quality control ensures fidelity in ribosome assembly and cellular health

• Published in: The Journal of cell biology Vol. 222; no. 4; p. 1
• Main Authors: Parker, Melissa D, Karbstein, Katrin
• Format: Journal Article
• Language: English
• Published: United States Rockefeller University Press 03.04.2023
• Subjects: Accuracy; Assembly; Biochemistry; Disease; Disease control; Fidelity; Gene expression; Homeostasis; Humans; Protein homeostasis; Proteins; Quality control; Review; Ribosomal Proteins - genetics; Ribosomal Proteins - metabolism; Ribosomal subunits; Ribosomes; Ribosomes - genetics; Ribosomes - metabolism; RNA biology; RNA, Ribosomal - metabolism; rRNA; Translation
• ISSN: 0021-9525; 1540-8140; 1540-8140
• DOI: 10.1083/jcb.202209115

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."