Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation.

ABSTRACT: Translation fidelity is crucial for prokaryotes and eukaryotic nuclear-encoded proteins; however, little is known about the role of mistranslation in mitochondria and its potential effects on metabolism. We generated yeast and mouse models with error-prone and hyper-accurate mitochondrial translation, and found that translation rate is more important than translational accuracy for cell function in mammals. Specifically, we found that mitochondrial mistranslation causes reduced overall mitochondrial translation and respiratory complex assembly rates. In mammals, this effect is compensated for by increased mitochondrial protein stability and upregulation of the citric acid cycle. Moreover, this induced mitochondrial stress signaling, which enables the recovery of mitochondrial translation v

SUBMITTER: Ferreira N 

PROVIDER: S-EPMC6912024 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature