Review www.cell-stress.com Mitochondrial dysfunction and its role in tissue-specific cellular stress David Pacheu-Grau1,*, Robert Rucktäschel1 and Markus Deckers1,* 1 Department of Cellular Biochemistry, University Medical Center Göttingen, Germany. * Corresponding Authors: David Pacheu-Grau, University Medical Center Göttingen, Department of Cellular Biochemistry, Humboldtallee 23, 37073 Göttingen, Germany. Phone: +49-(0)551-394571; E-mail:
[email protected]; Markus Deckers, University Medical Center Göttingen, Department of Cellular Biochemistry, Humboldtallee 23, 37073 Göttingen, Germany. Phone: +49-(0)551-395983; E-mail:
[email protected] ABSTRACT Mitochondrial bioenergetics require the coordination of two dif- ferent and independent genomes. Mutations in either genome will affect mi- Received originally: 26.04.2018 tochondrial functionality and produce different sources of cellular stress. De- in revised form: 13.06.2018, Accepted 14.06.2018, pending on the kind of defect and stress, different tissues and organs will be Published 13.07.2018. affected, leading to diverse pathological conditions. There is no curative ther- apy for mitochondrial diseases, nevertheless, there are strategies described that fight the various stress forms caused by the malfunctioning organelles. Keywords: mitochondrial dysfunction, Here, we will revise the main kinds of stress generated by mutations in mito- cellular stress, mitochondrial chondrial genes and outline several ways of fighting this stress. pathology, therapy. Abbreviations: ADOA – autosomal dominant optic atrophy, AROA – autosomal recessive optic atrophy, ARS – aminoacyl-tRNA synthetase, CL – cardiolipin, CRISPR – clustered regularly interspaced short palindromic repeats, LHON – Leber’s hereditary optic neuropathy, mt - mitochondrial OXPHOS – oxidative phosphorylation, ROS – reactive oxygen species. MITOCHONDRIA AND CELL METABOLISM like superoxide and hydrogen peroxide.