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The Mitochondrial Genome And Human Mitochondrial Diseases

Journal of the Peripheral Nervous System, 2002
To date, more than 100 point mutations and several hundreds of structural rearrangements of mitochondrial DNA (mtDNA) are known too be connected with characteristic neuromuscular and other mitochondrial syndromes varying from those causing death at the neonatal stage to diseases with late ages of onset. The immediate cause of mitochondrial disorders is
R I, Sukernik   +8 more
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Mitochondrial genome and longevity

Biochemistry (Moscow), 2016
The mitochondrial genome provides not only respiratory chain function, but it also ensures the impact of mitochondria on nearly all crucial metabolic processes. It is well known that mitochondria regulate aging and lifespan. However, until now there were no direct experimental data concerning the influence of various mitochondrial DNA variants on ...
R A, Zinovkin   +2 more
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The mitochondrial genome of yeast

Cell, 1978
mtDNA is the simplest DNA in nature which carries genes for the three classes of RNA-rRNAs, tRNAs, and mRNAs. In yeast, this genetic system has a number of unusual features that may even be of interest to molecular biologists who do not particularly care about mitochondrial biogenesis: -The gene for the large rRNA is split and the intervening sequence ...
P, Borst, L A, Grivell
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Deletions of the mitochondrial genome

Journal of Inherited Metabolic Disease, 1992
SummarySingle large deletions of mitochondrial DNA are found in the muscle of about 40% of patients with mitochondrial myopathies, and are detectable in both blood and muscle in Pearson syndrome. In mitochondrial myopathies, there is a close association between the presence of deletions and involvement of extra‐ocular muscles, together with other ...
A E, Harding, S R, Hammans
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Platyzoan mitochondrial genomes

Molecular Phylogenetics and Evolution, 2013
Platyzoa is a putative lophotrochozoan (spiralian) subtaxon within the protostome clade of Metazoa, comprising a range of biologically diverse, mostly small worm-shaped animals. The monophyly of Platyzoa, the relationships between the putative subgroups Platyhelminthes, Gastrotricha and Gnathifera (the latter comprising at least Gnathostomulida ...
Alexandra R, Wey-Fabrizius   +3 more
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Mitochondrial fusion and inheritance of the mitochondrial genome

Journal of Plant Research, 2010
Although maternal or uniparental inheritance of mitochondrial genomes is a general rule, biparental inheritance is sometimes observed in protists and fungi,including yeasts. In yeast, recombination occurs between the mitochondrial genomes inherited from both parents.Mitochondrial fusion observed in yeast zygotes is thought to set up a space for DNA ...
Hiroyoshi, Takano   +2 more
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The mitochondrial genome and mitochondrial muscle disorders

Current Opinion in Pharmacology, 2001
Mitochondrial disorders represent a multitude of clinically heterogeneous diseases in which the genetic abnormality can involve either a mitochondrial or nuclear gene. In addition to inherited defects, somatic mitochondrial DNA mutations have been implicated in the pathogenesis of neurodegenerative disease, cancer and the ageing process.
A M, Schaefer   +2 more
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The mitochondrial genome. The nucleoid

Biochemistry (Moscow), 2016
Mitochondrial DNA (mtDNA) in cells is organized in nucleoids containing DNA and various proteins. This review discusses questions of organization and structural dynamics of nucleoids as well as their protein components. The structures of mt-nucleoid from different organisms are compared.
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The influence of mitochondrial dynamics on mitochondrial genome stability

Current Genetics, 2017
Mitochondria are dynamic organelles that fuse and divide. These changes alter the number and distribution of mitochondrial structures throughout the cell in response to developmental and metabolic cues. We have demonstrated that mitochondrial fission is essential to the maintenance of mitochondrial DNA (mtDNA) under changing metabolic conditions in ...
Christopher T, Prevost   +6 more
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Mitochondrial genetics of Coprinus: Recombination of mitochondrial genomes

Current Genetics, 1983
The formation of the sexual mycelium or dikaryon in the basidiomycete Coprinus cinereus involves exchange and migration of nuclei without accompanying exchange of mitochondria. The dikaryotic growth which appears around the periphery of mated monokaryons has exclusively the mitochondrial genome of the recipient cells.
J L, Baptista-Ferreira   +2 more
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