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PXD015456 is an original dataset announced via ProteomeXchange.

Dataset Summary
TitleStress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation
DescriptionThe fidelity of translation is crucial in prokaryotes and for the nuclear-encoded proteins of eukaryotes, however little is known about the role of mistranslation in mitochondria and its effects on metabolism. We generated yeast and mouse models with error-prone and hyperaccurate mitochondrial translation fidelity and found that translation rate is more important than translational accuracy for cell function in mammals. We found that mitochondrial mistranslation reduces overall mitochondrial translation and the rate of respiratory complex assembly, however in mammals this is compensated for by increased mitochondrial protein stability and upregulation of the citric acid cycle. Moreover, mitochondrial stress signaling enables the recovery of mitochondrial translation via mitochondrial biogenesis, telomerase expression and cell proliferation, normalizing metabolism. Conversely, we show that increased fidelity of mitochondrial translation reduces the rate of protein synthesis without eliciting the mitochondrial stress response. Consequently, the rate of translation cannot be recovered causing dilated cardiomyopathy. Our findings reveal mammalian specific signaling pathways that can respond to changes in the fidelity of mitochondrial protein synthesis
ReviewLevelPeer-reviewed dataset
DatasetOriginOriginal dataset
RepositorySupportUnsupported dataset by repository
PrimarySubmitterIrina Kuznetsova
SpeciesList scientific name: Mus musculus (Mouse); NCBI TaxID: 10090;
ModificationListacetylated residue; monohydroxylated residue; iodoacetamide derivatized residue
InstrumentOrbitrap Fusion
Dataset History
RevisionDatetimeStatusChangeLog Entry
02019-09-16 01:12:13ID requested
12019-11-08 08:49:37announced
Publication List
Dataset with its publication pending
Keyword List
submitter keyword: metabolism, mitochondria, protein synthesis, stress response Abbreviations used: mitoribosome, mitochondrial ribosome
MRPS12, mitochondrial ribosomal protein of the small subunit 12
OXPHOS, oxidative phosphorylation
Contact List
Oliver Rackham
contact affiliation-Harry Perkins Institute of Medical Research, Nedlands, Western Australia 6009, Australia -School of Pharmacy and Biomedical Sciences, Curtin University, Bentley, Western Australia 6102, Australia -Curtin Health Innovation Research Institute, Curtin University, Bentley, WA 6102, Australia
contact emailoliver.rackham@curtin.edu.au
lab head
Irina Kuznetsova
contact affiliationUWA
contact emailirina.kuznetsova@perkins.org.au
dataset submitter
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Dataset FTP location
PRIDE project URI
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