The proteostasis network and its decline in ageing

MS Hipp, P Kasturi, FU Hartl - Nature reviews Molecular cell biology, 2019 - nature.com
Ageing is a major risk factor for the development of many diseases, prominently including
neurodegenerative disorders such as Alzheimer disease and Parkinson disease. A hallmark …

Ribosome-associated quality-control mechanisms from bacteria to humans

S Filbeck, F Cerullo, S Pfeffer, CAP Joazeiro - Molecular cell, 2022 - cell.com
Ribosome-associated quality-control (RQC) surveys incomplete nascent polypeptides
produced by interrupted translation. Central players in RQC are the human ribosome-and …

Ageing exacerbates ribosome pausing to disrupt cotranslational proteostasis

KC Stein, F Morales-Polanco, J van der Lienden… - Nature, 2022 - nature.com
Ageing is accompanied by a decline in cellular proteostasis, which underlies many age-
related protein misfolding diseases,. Yet, how ageing impairs proteostasis remains unclear …

In vivo aspects of protein folding and quality control

D Balchin, M Hayer-Hartl, FU Hartl - Science, 2016 - science.org
BACKGROUND Proteins are synthesized on ribosomes as linear chains of amino acids and
must fold into unique three-dimensional structures to fulfill their biological functions. Protein …

Mechanisms and functions of ribosome-associated protein quality control

CAP Joazeiro - Nature Reviews Molecular Cell Biology, 2019 - nature.com
The stalling of ribosomes during protein synthesis results in the production of truncated
polypeptides that can have deleterious effects on cells and therefore must be eliminated. In …

ZNF598 is a quality control sensor of collided ribosomes

S Juszkiewicz, V Chandrasekaran, Z Lin, S Kraatz… - Molecular cell, 2018 - cell.com
Aberrantly slow translation elicits quality control pathways initiated by the ubiquitin ligase
ZNF598. How ZNF598 discriminates physiologic from pathologic translation complexes and …

Protein misfolding diseases

FU Hartl - Annual review of biochemistry, 2017 - annualreviews.org
The majority of protein molecules must fold into defined three-dimensional structures to
acquire functional activity. However, protein chains can adopt a multitude of conformational …

Disome and trisome profiling reveal genome-wide targets of ribosome quality control

S Meydan, NR Guydosh - Molecular cell, 2020 - cell.com
The ribosome-associated protein quality control (RQC) system that resolves stalled
translation events is activated when ribosomes collide and form disome, trisome, or higher …

Ubiquitination of stalled ribosome triggers ribosome-associated quality control

Y Matsuo, K Ikeuchi, Y Saeki, S Iwasaki… - Nature …, 2017 - nature.com
Translation arrest by polybasic sequences induces ribosome stalling, and the arrest product
is degraded by the ribosome-mediated quality control (RQC) system. Here we report that …

Rethinking HSF1 in stress, development, and organismal health

J Li, J Labbadia, RI Morimoto - Trends in cell biology, 2017 - cell.com
The heat shock response (HSR) was originally discovered as a transcriptional response to
elevated temperature shock and led to the identification of heat shock proteins and heat …