The ribosome-bound quality control complex: from aberrant peptide clearance to proteostasis maintenance

Current Genetics
Quentin Defenouillère, Micheline Fromont-Racine

Abstract

Proteostasis in eukaryotes is maintained by compartment-specific quality control pathways, which enable the refolding or the degradation of defective polypeptides to prevent the toxicity that may arise from their aggregation. Among these processes, translational protein quality control is performed by the Ribosome-bound Quality Control complex (RQC), which recognizes nascent peptides translated from aberrant mRNAs, polyubiquitylates these aberrant peptides, extracts them from the stalled 60S subunit and finally escorts them to the proteasome for degradation. In this review, we focus on the mechanism of action of the RQC complex from stalled 60S binding to aberrant peptide delivery to the proteasome and describe the cellular consequences of a deficiency in the RQC pathway, such as aberrant protein aggregation. In addition, this review covers the recent discoveries concerning the role of cytosolic chaperones, as well as Tom1, to prevent the accumulation of aberrant protein aggregates in case of a deficiency in the RQC pathway.

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Citations

Jun 22, 2018·Annual Review of Biochemistry·Junhong ChoiJoseph D Puglisi
Nov 19, 2018·Current Genetics·Krishnamoorthi SrinivasanJayati Sengupta
Dec 5, 2018·Current Genetics·Carine Beaupere, Vyacheslav M Labunskyy
Feb 9, 2019·Nature Reviews. Molecular Cell Biology·Mark S HippF Ulrich Hartl
Apr 4, 2019·Nature Reviews. Molecular Cell Biology·Claudio A P Joazeiro
Dec 2, 2020·Molecular Cell·Caillan Crowe-McAuliffeVasili Hauryliuk
Dec 2, 2020·Molecular Cell·Sebastian FilbeckStefan Pfeffer
Apr 29, 2021·Molecular Cell·Anna ThrunClaudio A P Joazeiro
Aug 20, 2021·FEMS Microbiology Reviews·Colin R Harwood, Yoshimi Kikuchi

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Methods Mentioned

BETA
affinity purifications

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