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Mitochondrion

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https://www.readbyqxmd.com/read/28438612/glucose-and-glutamine-in-the-mitochondrial-oxidative-metabolism-of-stem-cells
#1
Salvatore Nesci
No abstract text is available yet for this article.
April 21, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28412540/melanopsin-expressing-retinal-ganglion-cells-are-resistant-to-cell-injury-but-not-always
#2
Birgitte Georg, Anna Ghelli, Carla Giordano, Fred N Ross-Cisneros, Alfredo A Sadun, Valerio Carelli, Jens Hannibal, Chiara La Morgia
Melanopsin retinal ganglion cells (mRGCs) are intrinsically photosensitive RGCs deputed to non-image forming functions of the eye such as synchronization of circadian rhythms to light-dark cycle. These cells are characterized by unique electrophysiological, anatomical and biochemical properties and are usually more resistant than conventional RGCs to different insults, such as axotomy and different paradigms of stress. We also demonstrated that these cells are relatively spared compared to conventional RGCs in mitochondrial optic neuropathies (Leber's hereditary optic neuropathy and Dominant Optic Atrophy)...
April 12, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28396254/mildly-deleterious-mitochondrial-dna-variants-are-less-frequent-in-healthy-older-individuals-but-not-more-prevalent-in-alzheimer-s-patients
#3
Ilse S Pienaar, Neil Howell, Joanna L Elson
Mitochondrial DNA (mtDNA) association studies have been conducted for over a decade using the haplogroup (lineage) association method, but this frequently produces conflicting results. Here we analyzed complete mtDNA sequence data of Alzheimer's disease (AD) patients and aged controls, from the United Kingdom (UK) and the United States (US), using a new "mutational load" method. We calculated a pathogenicity score for each of the non-synonymous substitutions of the mtDNA sequences to produce a "total mutational load" for each sequence, and compared the mutational loads of cases and controls...
April 7, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28396253/moderate-therapeutic-hypothermia-induces-multimodal-protective-effects-in-oxygen-glucose-deprivation-reperfusion-injured-cardiomyocytes
#4
Jana Krech, Giang Tong, Sylvia Wowro, Christoph Walker, Lisa-Maria Rosenthal, Felix Berger, Katharina Rose Luise Schmitt
OBJECTIVE: Therapeutic hypothermia has been shown to attenuate myocardial cell death due to ischemia/reperfusion injury. However, cellular mechanisms of cooling remain to be elucidated. Especially during reperfusion, mitochondrial dysfunction contributes to cell death by releasing apoptosis inductors. The aim of the present study was to investigate the effects of moderate therapeutic hypothermia (33.5°C) on mitochondrial mediated apoptosis in ischemia/reperfusion-injured cardiomyocytes...
April 7, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28365408/mitochondrial-dysfunction-underlying-outer-retinal-diseases
#5
Evy Lefevere, Anne Katrine Toft-Kehler, Rupali Vohra, Miriam Kolko, Lieve Moons, Inge Van Hove
Dysfunction of photoreceptors, retinal pigment epithelium (RPE) or both contribute to the initiation and progression of several outer retinal disorders. Disrupted Müller glia function might additionally subsidize to these diseases. Mitochondrial malfunctioning is importantly associated with outer retina pathologies, which can be classified as primary and secondary mitochondrial disorders. This review highlights the importance of oxidative stress and mitochondrial DNA damage, underlying outer retinal disorders...
March 29, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28351676/uncoupling-protein-2-and-metabolic-diseases
#6
REVIEW
Annapoorna Sreedhar, Yunfeng Zhao
Mitochondria are fascinating organelles involved in various cellular-metabolic activities that are integral for mammalian development. Although they perform diverse, yet interconnected functions, mitochondria are remarkably regulated by complex signaling networks. Therefore, it is not surprising that mitochondrial dysfunction is involved in plethora of diseases, including neurodegenerative and metabolic disorders. One of the many factors that lead to mitochondrial-associated metabolic diseases is the uncoupling protein-2, a family of mitochondrial anion proteins present in the inner mitochondrial membrane...
March 25, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28342934/mitochondrial-transplantation-from-animal-models-to-clinical-use-in-humans
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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/28025171/editorial
#17
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
#18
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
#19
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
#20
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
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