Elongational stalling activates mitoribosome-associated quality control

• Published in: Science (American Association for the Advancement of Science) Vol. 370; no. 6520; pp. 1105 - 1110
• Main Authors: Desai, Nirupa, Yang, Hanting, Chandrasekaran, Viswanathan, Kazi, Razina, Minczuk, Michal, Ramakrishnan, V
• Format: Journal Article
• Language: English
• Published: United States The American Association for the Advancement of Science 27.11.2020
• Subjects: Adenosine triphosphate; ATP; Chains; Cryoelectron Microscopy; Electron microscopy; Electron Transport Complex IV - chemistry; Elongated structure; Escherichia coli; Exoribonucleases - genetics; Genomes; HEK293 Cells; Homology; Humans; Intermediates; Metabolites; Microscopy; Mitochondria; Mitochondrial Proteins - chemistry; Mitochondrial Ribosomes - chemistry; Nuclear Proteins - chemistry; Oxidation; Oxidative phosphorylation; Peptide Termination Factors - chemistry; Phosphorylation; Polypeptides; Protein Domains; Proteins; Quality control; Ribonucleic acid; Ribosomal Proteins - chemistry; Ribosomes; RNA; RNA, Transfer - chemistry; RNA-binding protein; RNA-Binding Proteins - chemistry; Stalling; Synthesis; Transcription Elongation, Genetic; Transcriptional Elongation Factors - chemistry; Transfer RNA; Translation; Translation elongation; Translocase; Triphosphatase; tRNA

The human mitochondrial ribosome (mitoribosome) and associated proteins regulate the synthesis of 13 essential subunits of the oxidative phosphorylation complexes. We report the discovery of a mitoribosome-associated quality control pathway that responds to interruptions during elongation, and we pr...