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Mitochondrion

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https://www.readbyqxmd.com/read/28214560/mitochondrial-genotype-modulates-mtdna-copy-number-and-organismal-phenotype-in-drosophila
#1
Tiina S Salminen, Marcos T Oliveira, Giuseppe Cannino, Päivi Lillsunde, Howard T Jacobs, Laurie S Kaguni
We evaluated the role of natural mitochondrial DNA (mtDNA) variation on mtDNA copy number, biochemical features and life history traits in Drosophila cybrid strains. We demonstrate the effects of both coding region and non-coding A+T region variation on mtDNA copy number, and demonstrate that copy number correlates with mitochondrial biochemistry and metabolically important traits such as development time. For example, high mtDNA copy number correlates with longer development times. Our findings support the hypothesis that mtDNA copy number is modulated by mtDNA genome variation and suggest that it affects OXPHOS efficiency through changes in the organization of the respiratory membrane complexes to influence organismal phenotype...
February 15, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28216230/activation-of-a-cryptic-splice-site-in-the-mitochondrial-elongation-factor-gfm1-causes-combined-oxphos-deficiency
#2
Mariella T Simon, Bobby G Ng, Marisa W Friederich, Raymond Y Wang, Monica Boyer, Martin Kircher, Renata Collard, Kati J Buckingham, Richard Chang, Jay Shendure, Deborah A Nickerson, Michael J Bamshad, Johan L K Van Hove, Hudson H Freeze, Jose E Abdenur
We report the clinical, biochemical, and molecular findings in two brothers with encephalopathy and multi-systemic disease. Abnormal transferrin glycoforms were suggestive of a type I congenital disorder of glycosylation (CDG). While exome sequencing was negative for CDG related candidate genes, the testing revealed compound heterozygous mutations in the mitochondrial elongation factor G gene (GFM1). One of the mutations had been reported previously while the second, novel variant was found deep in intron 6, activating a cryptic splice site...
February 12, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28185966/disturbed-mitochondrial-function-restricts-glutamate-uptake-in-the-human-m%C3%A3-ller-glia-cell-line-mio-m1
#3
Rupali Vohra, Iswariyaraja Sridevi Gurubaran, Ulrik Henriksen, Linda Hildegaard Bergersen, Lene Juel Rasmussen, Claus Desler, Dorte Marie Skytt, Miriam Kolko
Using the human Müller cell line, MIO-M1, the aim was to study the impact of mitochondrial inhibition in Müller glia through antimycin A treatment. MIO-M1 cell survival, levels of released lactate, mitochondrial function, and glutamate uptake were studied in response to mitochondrial inhibition and glucose restriction. Lactate release decreased in response to glucose restriction. Combined glucose restriction and blocked mitochondrial activity decreased survival and caused collapse of the respiratory chain measured by oxygen consumption rate and extracellular acidification rate...
February 6, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28179130/mitochondrial-function-in-m%C3%A3-ller-cells-does-it-matter
#4
Anne Katrine Toft-Kehler, Dorte Marie Skytt, Alicia Svare, Evy Lefevere, Inge Van Hove, Lieve Moons, Helle S Waagepetersen, Miriam Kolko
Growing evidence suggests that mitochondrial dysfunction might play a key role in the pathogenesis of age-related neurodegenerative inner retinal diseases such as diabetic retinopathy and glaucoma. Therefore, the present review provides a perspective on the impact of functional mitochondria in the most predominant glial cells of the retina, the Müller cells. Müller cells span the entire thickness of the neuroretina and are in close proximity to retinal cells including the retinal neurons that provides visual signaling to the brain...
February 5, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28093355/evaluating-the-therapeutic-potential-of-idebenone-and-related-quinone-analogues-in-leber-hereditary-optic-neuropathy
#5
Patrick Yu-Wai-Man, Devorah Soiferman, David G Moore, Florence Burté, Ann Saada
Leber hereditary optic neuropathy (LHON) is an important cause of mitochondrial blindness among young adults. In this study, we investigated the potential of four quinone analogues (CoQ1, CoQ10, decylubiquinone and idebenone) in compensating for the deleterious effect of the m.11778G>A mitochondrial DNA mutation. The LHON fibroblast cell lines tested exhibited reduced cell growth, impaired mitochondrial bioenergetics and elevated levels of reactive oxygen species (ROS). Idebenone increased ATP production and reduced ROS levels, but the effect was partial and cell-specific...
January 16, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28093354/low-dose-resveratrol-ameliorates-mitochondrial-respiratory-dysfunction-and-enhances-cellular-reprogramming
#6
Yuki Mizuguchi, Hideyuki Hatakeyama, Kou Sueoka, Mamoru Tanaka, Yu-Ichi Goto
Mitochondrial disease is associated with a wide variety of clinical presentations, even among patients carrying heteroplasmic mitochondrial DNA (mtDNA) mutations, probably because of variations in mutant mtDNA proportions at the tissue and organ levels. Although several case reports and clinical trials have assessed the effectiveness of various types of drugs and supplements for the treatment of mitochondrial diseases, there are currently no cures for these conditions. In this study, we demonstrated for the first time that low dose resveratrol (RSV) ameliorated mitochondrial respiratory dysfunction in patient-derived fibroblasts carrying homoplasmic mtDNA mutations...
January 13, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28089944/rsm22-mtysxc-and-pnkd-like-proteins-are-required-for-mitochondrial-translation-in-trypanosoma-brucei
#7
Jiří Týč, Lucie Novotná, Priscilla Peña-Diaz, Dmitri A Maslov, Julius Lukeš
Mitochondrial ribosomes evolved from prokaryotic ribosomes, with which they therefore share more common features than with their counterparts in the cytosol. Yet, mitochondrial ribosomes are highly diverse in structure and composition, having undergone considerable changes, including reduction of their RNA component and varying degree of acquisition of novel proteins in various phylogenetic lineages. Here, we present functional analysis of three putative mitochondrial ribosome-associated proteins (RSM22, mtYsxC and PNKD-like) in Trypanosoma brucei, originally identified by database mining...
January 12, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28088649/fumarate-reductase-superfamily-a-diverse-group-of-enzymes-whose-evolution-is-correlated-to-the-establishment-of-different-metabolic-pathways
#8
Douglas Jardim-Messeder, Caroline Cabreira-Cagliari, Rafael Rauber, Andreia Carina Turchetto-Zolet, Rogério Margis, Márcia Margis-Pinheiro
Fumarate and succinate are known to be present in prebiotic systems essential for the origin of life. The fumarate and succinate interconversion reactions have been conserved throughout evolution and are found in all living organisms. The fumarate and succinate interconversion is catalyzed by the enzymes succinate dehydrogenase (SDH) and fumarate reductase (FRD). In this work we show that SDH and FRD are part of a group of enzymes that we propose to designate "fumarate reductase superfamily". Our results demonstrate that these enzymes emerged from a common ancestor and were essential in the development of metabolic pathways involved in energy transduction...
January 11, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28065674/mitochondria-in-teleost-spermatozoa
#9
REVIEW
Patricio Ulloa-Rodríguez, Elías Figueroa, Rommy Díaz, Manuel Lee-Estevez, Stefania Short, Jorge G Farías
There is an extraordinary diversity of reproductive modes in teleost and this variability is related to the phylogenetic relationships and adaption to very different biotopes. As in all vertebrates, sperm is produced as the end product of the process of spermatogenesis, and regarding teleost the spermatozoa lack an acrosome in almost all species and motility is activated as a response to osmolarity and ion content of the aquatic medium where the sperm is released. In this context, mitochondria possess a fundamental role for fish spermatozoa motility and integrity, hence, fertilizing potential; they are the energy supplier that allows flagellar movement and their dysfunction could play a main role in structural and functional damage to the spermatozoa...
January 5, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28025171/editorial
#10
EDITORIAL
Ian Max Møller, Jay J Thelen
No abstract text is available yet for this article.
December 23, 2016: Mitochondrion
https://www.readbyqxmd.com/read/28017685/physical-exercise-before-pregnancy-helps-the-development-of-mouse-embryos-produced-in-vitro
#11
Wen Hai Xu, Hao Wu, Wei Lan Xia, Hui Lan, Yongsheng Wang, Yong Zhang, Song Hua
Effects of pre-gestational physical activity on the later development of embryos generated in vitro were evaluated. Kunming mice were divided into two groups, namely exercised and unexercised, with the former undergoing physical training on a motor-driven leveled treadmill over a period of 4weeks (5days/week and 60min/day). After that, following superovulation, collection of oocytes from both groups was performed for in vitro fertilization (IVF) and somatic cell nuclear transfer (SCNT). Notably and specifically, natural mating between the unexercised mice was also done for in vivo fertilization (IVIF)...
December 23, 2016: Mitochondrion
https://www.readbyqxmd.com/read/28017684/increased-levels-of-cell-free-mitochondrial-dna-in-the-cerebrospinal-fluid-of-patients-with-multiple-sclerosis
#12
Kristin N Varhaug, Christian A Vedeler, Kjell-Morten Myhr, Jan Harald Aarseth, Charalampos Tzoulis, Laurence A Bindoff
Mitochondrial DNA (mtDNA) can act as damage-associated molecular pattern molecule (DAMP) and initiate an inflammatory response. We hypothesized that the concentration of mtDNA might reflect inflammatory activity in multiple sclerosis and investigated therefore levels of cell-free mitochondrial DNA in cerebrospinal fluid of patients with relapsing-remitting multiple sclerosis. Significantly higher levels of mtDNA were found in patients compared to controls and there was an inverse correlation between disease duration and mtDNA concentration...
December 23, 2016: Mitochondrion
https://www.readbyqxmd.com/read/28007605/mitochondrial-networking-in-diabetic-left-ventricle-cardiomyocytes
#13
REVIEW
Lucia-Doina Popov
Cardiomyocyte mitochondria preserve "the quorum sensing" attribute of their aerobic bacterial ancestors, as shown by the transient physical connectivity and communication not only with each other, but also with other intracellular organelles and with cytosol, ensuing cellular homeostasis. In this review, we present original electron microscopy evidence on mitochondrial networking within diabetic left ventricular cardiomyocytes, focusing on: (i) the inter-mitochondrial communication, allowing electrochemical signals transfer and outer membrane components or matrix proteins exchange, (ii) the interplay between mitochondria and the cardiomyocyte nucleus, nucleolus, sarcoplasmic reticulum, lysosomes, and lipid droplets viewed as attributes of mitochondrial "quality control" and "retrograde signaling function", and (iii) the crosstalk between mitochondria and cardiomyocyte cytosol, as part of the adaptive responses that allow cells survival...
December 19, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27979772/integration-of-mtdna-pseudogenes-into-the-nuclear-genome-coincides-with-speciation-of-the-human-genus-a-hypothesis
#14
Konstantin Gunbin, Leonid Peshkin, Konstantin Popadin, Sofia Annis, Rebecca R Ackermann, Konstantin Khrapko
Fragments of mitochondrial DNA are known to get inserted into nuclear DNA to form NUMTs, i.e. nuclear pseudogenes of the mtDNA. The insertion of a NUMT is a rare event. Hundreds of pseudogenes have been cataloged in the human genome. NUMTs are, in essence, a special type of mutation with their own internal timer, which is synchronized with an established molecular clock, the mtDNA. Thus insertion of NUMTs can be timed with respect to evolution milestones such as the emergence of new species. We asked whether NUMTs were inserted uniformly over time or preferentially during certain periods of evolution, as implied by the "punctuated evolution" model...
December 12, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27838478/random-shearing-as-an-alternative-to-digestion-for-mitochondrial-dna-processing-in-droplet-digital-pcr
#15
Andrej Vitomirov, Miguel Ramirez-Gaona, Sanjay R Mehta, Josué Pérez-Santiago
Droplet digital PCR (ddPCR) is a quantitative assay that requires DNA fragmentation to maximize reaction efficiency. Here, we measured the proportion of mitochondrial DNA (mtDNA) carrying the "common deletion," a rare event, to compare quantification sensitivities between alternative DNA fragmentation methods (sonication and QIAshredder spin columns) against enzymatic digestion (traditionally used). QIAshredder showed the highest sensitivity when compared to sonication, followed by digestion. Also, both sonication and QIAshredder fragmentation had shorter processing times than enzymatic digestion; therefore, QIAshredder fragmentation and sonication are alternative DNA processing methods that maximize ddPCR quantification for the detection of rare events...
January 2017: Mitochondrion
https://www.readbyqxmd.com/read/27890822/visual-light-effects-on-mitochondria-the-potential-implications-in-relation-to-glaucoma
#16
Neville N Osborne, Claudia Núñez-Álvarez, Susana Del Olmo-Aguado, Jesús Merrayo-Lloves
Light of different wave-lengths have the potential to interact with four major mitochondrial protein complexes that are involved in the generation of ATP. Neurones of the central nervous system have an absolute dependence on mitochondrial generated ATP. Laboratory studies show that short-wave or blue light (400-480nm) that impinges on the retina affect flavin and cytochrome constituents associated with mitochondria to decrease the rate of ATP formation, stimulate ROS and results in cell death. This suggests that blue light could potentially have a negative influence on retinal ganglion cell (RGC) mitochondria that are abundant and not shielded by macular pigments as occurs for photoreceptor mitochondria...
November 24, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27865797/radboud-centre-for-mitochondrial-medicine-pediatric-mri-score
#17
Sheila Suet-Na Wong, Bozena Goraj, Cheuk-Wing Fung, Jeroen Vister, Lonneke de Boer, Saskia Koene, Jan Smeitink
We developed the first user-friendly, semi-quantitative, and quick-to-perform Radboud Centre for Mitochondrial Medicine Pediatric MRI score (RCMM-PMRIS), focusing on the six most commonly described neuroimaging abnormalities in the literature. The RCMM-PMRIS was validated through individual review of 30 sets of brain MRI studies in 24 patients with genetically confirmed mitochondrial disorders by six raters. The application of RCMM-PMRIS can help to define the extent of the brain involvement and therefore to assess the radiological mitochondrial disease severity, to monitor disease progression and consequently to act as an outcome measure for treatment effects in patients with mitochondrial disease...
November 16, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27856367/dysphagia-is-prevalent-in-patients-with-cpeo-and-single-large-scale-deletions-in-mtdna
#18
Gitte Hedermann, Nicoline Løkken, Julia R Dahlqvist, John Vissing
BACKGROUND: The aim of this study was to assess the frequency of subjective and objective dysphagia in patients with chronic progressive external ophthalmoplegia (CPEO) due to single, large-scale deletions (LSDs) of mitochondrial DNA (mtDNA). METHODS: Sixteen patients with CPEO and single LSDs of mtDNA were included in the study and compared to a control group of 12 patients with the m.3243A>G mtDNA mutation. Patients had to drink 80ml of water at 4°C as fast as they could (cold-water test) and fill out a standardized questionnaire about dysphagia...
November 14, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27847334/investigating-leber-s-hereditary-optic-neuropathy-cell-models-and-future-perspectives
#19
REVIEW
Elona Jankauskaitė, Ewa Bartnik, Agata Kodroń
Leber's hereditary optic neuropathy (LHON) was the first human disease found to be associated with a mitochondrial DNA (mtDNA) point mutation. The most common LHON mutations are 11778G>A, 3460G>A or 14484T>C. The most common clinical features of LHON are optic nerve and retina atrophy. The affected tissue is not available for studies, therefore a variety of other cell types are used. However, all models face difficulties and limitations in mitochondrial disease research. The advantages and disadvantages of different cell models used to study LHON, recent advances in animal model generation and novel approaches in this field are discussed...
November 12, 2016: Mitochondrion
https://www.readbyqxmd.com/read/27845271/characterizing-the-mitochondrial-dna-polymerase-gamma-interactome-by-bioid-identifies-ruvbl2-localizes-to-the-mitochondria
#20
Sanduni U Liyanage, Etienne Coyaud, Estelle M N Laurent, Rose Hurren, Neil Maclean, Stuart R Wood, Lawrence Kazak, Aisha Shamas-Din, Ian Holt, Brian Raught, Aaron Schimmer
Human mitochondrial DNA (mtDNA) is replicated by the mitochondrial DNA polymerase gamma (POLG). Using proximity dependent biotin labelling (BioID), we characterized the POLG interactome and identified new interaction partners involved in mtDNA maintenance, transcription, translation and protein quality control. We also identified interaction with the nuclear AAA+ ATPase Ruvbl2, suggesting mitochondrial localization for this protein. Ruvbl2 was detected in mitochondria-enriched fractions in leukemic cells. Additionally, transgenic overexpression of Ruvbl2 from an alternative translation initiation site resulted in mitochondrial co-localization...
November 11, 2016: Mitochondrion
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