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Mitochondrion

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https://www.readbyqxmd.com/read/28342934/mitochondrial-transplantation-from-animal-models-to-clinical-use-in-humans
#1
REVIEW
James D McCully, Douglas B Cowan, Sitaram M Emani, Pedro J Del Nido
Mitochondrial transplantation is a novel therapeutic intervention to treat ischemia/reperfusion related disorders. The method for mitochondrial transplantation is simple and rapid and can be delivered to the end organ either by direct injection or vascular infusion. In this review, we provide mechanistic and histological studies in large animal models and present data to show clinical efficacy in human patients.
March 22, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28288917/cyclophilin-d-regulates-lifespan-and-protein-expression-of-aging-markers-in-the-brain-of-mice
#2
Viktoria Vereczki, Josef Mansour, Issa Pour-Ghaz, Ibolya Bodnar, Otto Pinter, Dora Zelena, Erzsebet Oszwald, Vera Adam-Vizi, Christos Chinopoulos
Cyclophilin D (cypD) modulates the properties of the permeability transition pore, a phenomenon implicated in the manifestation of many diseases including aging. Here, we examined the effects of partial or complete deletion of cypD on i) lifespan, ii) forebrain protein expression of 18 aging markers as well as regional expression of GFAP, mGluR1, and alpha-synuclein, and iii) behaviour of aged (>24month) male and female mice. Both male and female cypD heterozygous but not KO mice exhibited increased lifespans compared to WT littermates, associated with alterations in the protein expression of some markers, albeit without exhibiting changes in behaviour...
March 10, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28286264/optical-coherence-tomography-angiography-of-the-peripapillary-retina-and-optic-nerve-head-in-dominant-optic-atrophy
#3
Nicole Balducci, Antonio Ciardella, Roberto Gattegna, Qienyuan Zhou, Maria Lucia Cascavilla, Chiara La Morgia, Giacomo Savini, Vincenzo Parisi, Francesco Bandello, Valerio Carelli, Piero Barboni
Peripapillar and nerve head vessel density (VD) was measured in 10 patients affected by Dominant optic atrophy (DOA) using optical coherence tomography angiography (OCT-A) and compared to the measurements of 15 age- and gender-matched controls. DOA patients showed VD reduction, mostly in the temporal and inferotemporal peripapillary sectors, according to the preferential involvement of the papillomacular bundle. Despite poor best-corrected visual acuity (BCVA), OCT-A revealed good repeatability. VD correlated with functional (mean deviation of visual field and BCVA) and structural (retinal nerve fiber layer thickness) parameters and could be a non-invasive, quantitative tool for the monitoring of the disease and of the therapeutic approaches...
March 10, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28263872/evidence-of-oxidative-stress-and-mitochondrial-dysfunction-in-spinocerebellar-ataxia-type-2-sca2-patient-fibroblasts-effect-of-coenzyme-q10-supplementation-on-these-parameters
#4
Nanna Cornelius, Jonathan H Wardman, Iain P Hargreaves, Viruna Neergheen, Anne Sigaard Bie, Zeynep Tümer, Jørgen E Nielsen, Troels T Nielsen
Spinocerebellar ataxia type 2 (SCA2) is a rare neurodegenerative disorder caused by a CAG repeat expansion in the ataxin-2 gene. We show increased oxidative stress, abnormalities in the antioxidant system, changes in complexes involved in oxidative phosphorylation and changes in mitochondrial morphology in SCA2 patient fibroblasts compared to controls, and we show that treatment with CoQ10 can partially reverse these changes. Together, our results suggest that oxidative stress and mitochondrial dysfunction may be contributory factors to the pathophysiology of SCA2 and that therapeutic strategies involving manipulation of the antioxidant system could prove to be of clinical benefit...
March 3, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28263873/liver-transplant-reverses-biochemical-imbalance-in-mitochondrial-neurogastrointestinal-encephalomyopathy
#5
LETTER
Roberto D'Angelo, Rita Rinaldi, Loris Pironi, Maria Teresa Dotti, Antonio Daniele Pinna, Elisa Boschetti, Mariantonietta Capristo, Susan Mohamed, Manuela Contin, Leonardo Caporali, Valerio Carelli, Roberto De Giorgio
No abstract text is available yet for this article.
March 2, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28242362/intravenous-administration-of-mitochondria-for-treating-experimental-parkinson-s-disease
#6
Xianxun Shi, Ming Zhao, Chen Fu, Ailing Fu
Mitochondrial dysfunction is associated with a large number of human diseases, including neurological and muscular degeneration, cardiovascular disorders, obesity, diabetes, aging and rare mitochondrial diseases. Replacement of dysfunctional mitochondria with functional exogenous mitochondria is proposed as a general principle to treat these diseases. Here we found that mitochondria isolated from human hepatoma cell could naturally enter human neuroblastoma SH-SY5Y cell line, and when the mitochondria were intravenously injected into mice, all of the mice were survived and no obvious abnormality appeared...
February 24, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28214560/mitochondrial-genotype-modulates-mtdna-copy-number-and-organismal-phenotype-in-drosophila
#7
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 16, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28216230/activation-of-a-cryptic-splice-site-in-the-mitochondrial-elongation-factor-gfm1-causes-combined-oxphos-deficiency
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
EDITORIAL
Ian Max Møller, Jay J Thelen
No abstract text is available yet for this article.
March 2017: Mitochondrion
https://www.readbyqxmd.com/read/27592226/the-mitochondrial-bkca-channel-cardiac-interactome-reveals-bkca-association-with-the-mitochondrial-import-receptor-subunit-tom22-and-the-adenine-nucleotide-translocator
#17
Jin Zhang, Min Li, Zhu Zhang, Ronghui Zhu, Riccardo Olcese, Enrico Stefani, Ligia Toro
Mitochondrial BKCa channel, mitoBKCa, regulates mitochondria function in the heart but information on its protein partnerships in cardiac mitochondria is missing. A directed proteomic approach discovered the novel interaction of BKCa with Tom22, a component of the mitochondrion outer membrane import system, and the adenine nucleotide translocator (ANT). The expressed protein partners co-immunoprecipitated and co-segregated into mitochondrial fractions in HEK293T cells. The BKCa 50 amino acid splice insert, DEC, facilitated BKCa interaction with ANT...
March 2017: Mitochondrion
https://www.readbyqxmd.com/read/27535110/the-proteome-of-baker-s-yeast-mitochondria
#18
Humberto Gonczarowska-Jorge, René P Zahedi, Albert Sickmann
In the past decade mass spectrometry-based proteomics has greatly contributed to shaping our knowledge about Saccharomyces cerevisiae mitochondria, from the initial identification of novel essential components in purified protein complexes, to the actual characterization of the mitochondrial proteome, the specific analysis of mitochondrial subcompartment proteomes, and the study of regulatory mechanisms that govern mitochondrial homeostasis. Here, we provide an overview of relevant mitochondrial proteome studies and furthermore discuss future possibilities how proteomics will further improve our existing understanding of mitochondria...
March 2017: Mitochondrion
https://www.readbyqxmd.com/read/27521611/mitochondrial-phosphoproteomics-of-mammalian-tissues
#19
Rikke Kruse, Kurt Højlund
Mitochondria are essential for several biological processes including energy metabolism and cell survival. Accordingly, impaired mitochondrial function is involved in a wide range of human pathologies including diabetes, cancer, cardiovascular, and neurodegenerative diseases. Within the past decade a growing body of evidence indicates that reversible phosphorylation plays an important role in the regulation of a variety of mitochondrial processes as well as tissue-specific mitochondrial functions in mammals...
March 2017: Mitochondrion
https://www.readbyqxmd.com/read/27476757/lysine-acetylation-in-mitochondria-from-inventory-to-function
#20
Fabian Hosp, Ines Lassowskat, Valeria Santoro, David De Vleesschauwer, Daniela Fliegner, Henning Redestig, Matthias Mann, Sven Christian, Matthew A Hannah, Iris Finkemeier
Cellular signaling pathways are regulated in a highly dynamic fashion in order to quickly adapt to distinct environmental conditions. Acetylation of lysine residues represents a central process that orchestrates cellular metabolism and signaling. In mitochondria, acetylation seems to be the most prevalent post-translational modification, presumably linked to the compartmentation and high turnover of acetyl-CoA in this organelle. Similarly, the elevated pH and the higher concentration of metabolites in mitochondria seem to favor non-enzymatic lysine modifications, as well as other acylations...
March 2017: Mitochondrion
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