Quality control of the mitochondrial proteome

J Song, JM Herrmann, T Becker - Nature Reviews Molecular Cell …, 2021 - nature.com
Abstract Mitochondria contain about 1,000–1,500 proteins that fulfil multiple functions.
Mitochondrial proteins originate from two genomes: mitochondrial and nuclear. Hence …

Mechanisms and regulation of protein synthesis in mitochondria

E Kummer, N Ban - Nature Reviews Molecular Cell Biology, 2021 - nature.com
Mitochondria are cellular organelles responsible for generation of chemical energy in the
process called oxidative phosphorylation. They originate from a bacterial ancestor and …

Mitochondrial stress is relayed to the cytosol by an OMA1–DELE1–HRI pathway

X Guo, G Aviles, Y Liu, R Tian, BA Unger, YHT Lin… - Nature, 2020 - nature.com
In mammalian cells, mitochondrial dysfunction triggers the integrated stress response, in
which the phosphorylation of eukaryotic translation initiation factor 2α (eIF2α) results in the …

[HTML][HTML] Quality control of the mitochondrion

MYW Ng, T Wai, A Simonsen - Developmental cell, 2021 - cell.com
Mitochondria are essential organelles that execute and coordinate various metabolic
processes in the cell. Mitochondrial dysfunction severely affects cell fitness and contributes …

A cytosolic surveillance mechanism activates the mitochondrial UPR

FXR Sutandy, I Gößner, G Tascher, C Münch - Nature, 2023 - nature.com
The mitochondrial unfolded protein response (UPRmt) is essential to safeguard
mitochondria from proteotoxic damage by activating a dedicated transcriptional response in …

SLC25A39 is necessary for mitochondrial glutathione import in mammalian cells

Y Wang, FS Yen, XG Zhu, RC Timson, R Weber, C Xing… - Nature, 2021 - nature.com
Glutathione (GSH) is a small-molecule thiol that is abundant in all eukaryotes and has key
roles in oxidative metabolism. Mitochondria, as the major site of oxidative reactions, must …

Mitophagy and quality control mechanisms in mitochondrial maintenance

S Pickles, P Vigié, RJ Youle - Current Biology, 2018 - cell.com
The maintenance of a healthy and functional mitochondrial network is critical during
development as well as throughout life in the response to physiological adaptations and …

A pathway coordinated by DELE1 relays mitochondrial stress to the cytosol

E Fessler, EM Eckl, S Schmitt, IA Mancilla… - Nature, 2020 - nature.com
Mitochondrial fidelity is tightly linked to overall cellular homeostasis and is compromised in
ageing and various pathologies,–. Mitochondrial malfunction needs to be relayed to the …

Building and decoding ubiquitin chains for mitophagy

JW Harper, A Ordureau, JM Heo - Nature reviews Molecular cell biology, 2018 - nature.com
Mitochondria produce energy in the form of ATP via oxidative phosphorylation. As defects in
oxidative phosphorylation can generate harmful reactive oxygen species, it is important that …

Enhancing mitochondrial proteostasis reduces amyloid-β proteotoxicity

V Sorrentino, M Romani, L Mouchiroud, JS Beck… - Nature, 2017 - nature.com
Alzheimer's disease is a common and devastating disease characterized by aggregation of
the amyloid-β peptide. However, we know relatively little about the underlying molecular …