Mitochondrial diseases
GS Gorman, PF Chinnery, S DiMauro… - Nature reviews Disease …, 2016 - nature.com
Mitochondrial diseases are a group of genetic disorders that are characterized by defects in
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …
Maintenance and expression of mammalian mitochondrial DNA
CM Gustafsson, M Falkenberg… - Annual review of …, 2016 - annualreviews.org
Mammalian mitochondrial DNA (mtDNA) encodes 13 proteins that are essential for the
function of the oxidative phosphorylation system, which is composed of four respiratory …
function of the oxidative phosphorylation system, which is composed of four respiratory …
Base excision repair
HE Krokan, M Bjørås - Cold Spring Harbor perspectives …, 2013 - cshperspectives.cshlp.org
Base excision repair (BER) corrects DNA damage from oxidation, deamination and
alkylation. Such base lesions cause little distortion to the DNA helix structure. BER is …
alkylation. Such base lesions cause little distortion to the DNA helix structure. BER is …
Plant mitochondrial genomes: dynamics and mechanisms of mutation
JM Gualberto, KJ Newton - Annual review of plant biology, 2017 - annualreviews.org
The large mitochondrial genomes of angiosperms are unusually dynamic because of
recombination activities involving repeated sequences. These activities generate …
recombination activities involving repeated sequences. These activities generate …
The maintenance of mitochondrial DNA integrity—critical analysis and update
M Alexeyev, I Shokolenko… - Cold Spring Harbor …, 2013 - cshperspectives.cshlp.org
DNA molecules in mitochondria, just like those in the nucleus of eukaryotic cells, are
constantly damaged by noxious agents. Eukaryotic cells have developed efficient …
constantly damaged by noxious agents. Eukaryotic cells have developed efficient …
Minimizing the damage: repair pathways keep mitochondrial DNA intact
Mitochondrial DNA (mtDNA) faces the universal challenges of genome maintenance: the
accurate replication, transmission and preservation of its integrity throughout the life of the …
accurate replication, transmission and preservation of its integrity throughout the life of the …
[HTML][HTML] Linear mitochondrial DNA is rapidly degraded by components of the replication machinery
Emerging gene therapy approaches that aim to eliminate pathogenic mutations of
mitochondrial DNA (mtDNA) rely on efficient degradation of linearized mtDNA, but the …
mitochondrial DNA (mtDNA) rely on efficient degradation of linearized mtDNA, but the …
DNA helicases involved in DNA repair and their roles in cancer
RM Brosh Jr - Nature Reviews Cancer, 2013 - nature.com
Helicases have major roles in genome maintenance by unwinding structured nucleic acids.
Their prominence is marked by various cancers and genetic disorders that are linked to …
Their prominence is marked by various cancers and genetic disorders that are linked to …
Somatic mitochondrial DNA mutations in mammalian aging
NG Larsson - Annual review of biochemistry, 2010 - annualreviews.org
Mitochondrial dysfunction is heavily implicated in the multifactorial aging process. Aging
humans have increased levels of somatic mtDNA mutations that tend to undergo clonal …
humans have increased levels of somatic mtDNA mutations that tend to undergo clonal …
Mechanism of the ATP-dependent DNA end-resection machinery from Saccharomyces cerevisiae
If not properly processed and repaired, DNA double-strand breaks (DSBs) can give rise to
deleterious chromosome rearrangements, which could ultimately lead to the tumour …
deleterious chromosome rearrangements, which could ultimately lead to the tumour …