Mitochondrial respiratory dysfunction-elicited oxidative stress and posttranslational protein modification in mitochondrial diseases

Annals of the New York Academy of Sciences
Yu-Ting WuYau-Huei Wei

Abstract

Pathogenic mutation in mtDNA and mitochondrial dysfunction are associated with mitochondrial diseases. In this review, we discuss the oxidative stress-elicited mitochondrial protein modifications that may contribute to the pathophysiology of mitochondrial diseases. We demonstrated that excess ROS produced by defective mitochondria could increase the acetylation of microtubule proteins through the suppression of Sirt2, which results in perinuclear distribution of mitochondria in skin fibroblasts of patients with CPEO syndrome. Our recent work showed that mitochondrial dysfunction-induced oxidative stress can disrupt protein degradation system by inhibiting the ubiquitin-proteasome pathway and protease activity in human cells harboring mutant mtDNA. This in turn causes accumulation of aberrant proteins in mitochondria and renders the mutant cells more susceptible to apoptosis induced by oxidative stress. Furthermore, oxidative stress can modulate phosphorylation of mitochondrial proteins, which can affect metabolism in a number of diseases. Taken together, we suggest that oxidative stress-triggered protein modifications and defects in protein turnover play an important role in the pathogenesis and progression of mitochondrial dis...Continue Reading

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Citations

Dec 1, 2011·Acta Neuropsychiatrica·Fabiano R AgostinhoJoão Quevedo
May 29, 2012·Clinical Medicine & Research·Eric R AndersonMichael D Caldwell
Sep 30, 2014·Genes·Filipe V DuarteAnabela P Rolo
Nov 13, 2012·Toxicology and Applied Pharmacology·Marco PelinAurelia Tubaro
May 11, 2015·Biochemical and Biophysical Research Communications·Yuki MiyamotoJunji Yamauchi
Apr 24, 2014·TheScientificWorldJournal·Daniela SorrientoGuido Iaccarino
Mar 26, 2013·Toxicology Letters·Rawabi QadhiJohn M Seubert
Mar 25, 2015·Scientific Reports·Antonella Marino GammazzaAlberto J L Macario
Nov 11, 2020·Acta Neuropathologica Communications·Peter M J QuinnC Henrique Alves
Jan 8, 2021·Molecular Neurobiology·G MorrisB K Puri

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