Mitochondrial DNA damage and repair in neurodegenerative disorders
نویسندگان
چکیده
منابع مشابه
Mitochondrial DNA repair and damage tolerance.
The accurate maintenance of mitochondrial DNA (mtDNA) is required in order for eukaryotic cells to assemble a functional electron transport chain. This independently-maintained genome relies on nuclear-encoded proteins that are imported into the mitochondria to carry out replication and repair processes. Decades of research has made clear that mitochondria employ robust and varied mtDNA repair ...
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There is compelling evidence for the direct involvement of mitochondria in certain neurodegenerative disorders, such as Morbus Parkinson, FRDA (Friedreich's ataxia), ALS (amyotrophic lateral sclerosis), and temporal lobe epilepsy with Ammon's horn sclerosis. This evidence includes the direct genetic evidence of pathogenic mutations in mitochondrial proteins in inherited Parkinsonism {such as PA...
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Mitochondria contain the only extra-nuclear source of DNA. Under evolutionary pressure mitochondrial DNA (mtDNA) has adapted from genomes containing over 1,000 kb containing significant quantities of non-coding DNA to the highly compact mammalian mtDNA. In humans, the mitochondrial genome consists of a small (16.5 kb) double-stranded circular genome constituting less than 1% of the total cellul...
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Background: Single nucleotide polymorphisms in 8-oxoguanine DNA glycosylase-1 (OGG1) gene modulates DNA repair capacity and functions as one of the first lines of protective mechanisms against 8-hydroxy-2’-deoxyguanosine (8-OHdG) mutagenicity. OGG1-Cys326 gene polymorphism may decrease DNA repair function, causing oxidative stress due to higher oxidative DNA damage. The main purpose of this stu...
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Defects in cellular DNA repair processes have been linked to genome instability, heritable cancers, and premature aging syndromes. Yet defects in some repair processes manifest themselves primarily in neuronal tissues. This review focuses on studies defining the molecular defects associated with several human neurological disorders, particularly ataxia with oculomotor apraxia 1 (AOA1) and spino...
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ژورنال
عنوان ژورنال: DNA Repair
سال: 2008
ISSN: 1568-7864
DOI: 10.1016/j.dnarep.2008.03.012