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Mitochondrial DNA damage and reactive oxygen species in neurodegenerative disease
Author(s) -
Nissanka Nadee,
Moraes Carlos T.
Publication year - 2018
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1002/1873-3468.12956
Subject(s) - neurodegeneration , mitochondrial dna , mitochondrion , reactive oxygen species , dna damage , biology , oxidative phosphorylation , dna oxidation , microbiology and biotechnology , mitochondrial ros , dna , biochemistry , gene , disease , pathology , medicine
Mitochondria are essential organelles within the cell where most ATP is produced through oxidative phosphorylation ( OXPHOS ). A subset of the genes needed for this process are encoded by the mitochondrial DNA (mt DNA ). One consequence of OXPHOS is the production of mitochondrial reactive oxygen species ( ROS ), whose role in mediating cellular damage, particularly in damaging mt DNA during ageing, has been controversial. There are subsets of neurons that appear to be more sensitive to ROS ‐induced damage, and mitochondrial dysfunction has been associated with several neurodegenerative disorders. In this review, we will discuss the current knowledge in the field of mt DNA and neurodegeneration, the debate about ROS as a pathological or beneficial contributor to neuronal function, bona fide mt DNA diseases, and insights from mouse models of mt DNA defects affecting the central nervous system.