Journal ArticleDOI
Quality control of the mitochondrial proteome.
TLDR
To ensure proper mitochondrial function, cells use multiple mechanisms of quality control that survey mitochondrial protein biogenesis, import and folding, and allow mitochondria to adapt to the changing needs as well as to respond to stresses that compromise proteostasis.Abstract:
Mitochondria contain about 1,000-1,500 proteins that fulfil multiple functions. Mitochondrial proteins originate from two genomes: mitochondrial and nuclear. Hence, proper mitochondrial function requires synchronization of gene expression in the nucleus and in mitochondria and necessitates efficient import of mitochondrial proteins into the organelle from the cytosol. Furthermore, the mitochondrial proteome displays high plasticity to allow the adaptation of mitochondrial function to cellular requirements. Maintenance of this complex and adaptable mitochondrial proteome is challenging, but is of crucial importance to cell function. Defects in mitochondrial proteostasis lead to proteotoxic insults and eventually cell death. Different quality control systems monitor the mitochondrial proteome. The cytosolic ubiquitin-proteasome system controls protein transport across the mitochondrial outer membrane and removes damaged or mislocalized proteins. Concomitantly, a number of mitochondrial chaperones and proteases govern protein folding and degrade damaged proteins inside mitochondria. The quality control factors also regulate processing and turnover of native proteins to control protein import, mitochondrial metabolism, signalling cascades, mitochondrial dynamics and lipid biogenesis, further ensuring proper function of mitochondria. Thus, mitochondrial protein quality control mechanisms are of pivotal importance to integrate mitochondria into the cellular environment.read more
Citations
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Molecular basis of selective mitochondrial fusion by heterotypic action between OPA1 and cardiolipin
Tadato Ban,Naotada Ishihara +1 more
TL;DR: In this paper, the authors showed that L-OPA1 and cardiolipin (CL) cooperate in heterotypic mitochondrial inner membrane (IM) fusion and cristae morphology.
Journal ArticleDOI
Mechanisms and regulation of protein synthesis in mitochondria
Eva Kummer,Nenad Ban +1 more
TL;DR: In this paper, the authors review fundamental insights that have been made into the biogenesis, architecture and mechanisms of the mitochondrial translation apparatus in the past years owing to the emergence of numerous near-atomic structures and a considerable amount of biochemical work.
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Signaling pathways and defense mechanisms of ferroptosis.
Jiao Liu,Rui Kang,Daolin Tang +2 more
TL;DR: In this paper, the authors summarize recent progress in understanding the signaling pathways and defense mechanisms of ferroptosis and summarize the main nuclear mechanism of cell defense against ferro-ptosis by transcriptionally upregulating the expression of antioxidants or cytoprotective genes.
Journal ArticleDOI
Beyond K48 and K63: non-canonical protein ubiquitination
Michał Tracz,Wojciech Bialek +1 more
TL;DR: A concise summary of recent advances in the field of poorly understood ubiquitin linkages and their possible roles in vivo can be found in this paper, where the authors provide a concise summary.
Cytosolic protein Vms1 links ribosome quality control to mitochondrial and cellular homeostasis
Toshiaki Izawa,Toshiaki Izawa,Sae Hun Park,Liang Zhao,F. Ulrich Hartl,Walter Neupert,Walter Neupert +6 more
TL;DR: In this article, the authors show that yeast cells continually produce faulty mitochondrial polypeptides that stall on the ribosome during translation but are imported into the mitochondria, and that the cytosolic protein Vms1, together with the E3 ligase Ltn1, protects against the mitochondrial toxicity of these proteins and maintains cell viability under respiratory conditions.
References
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