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Mitochondrion

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https://www.readbyqxmd.com/read/28533168/prospects-for-therapeutic-mitochondrial-transplantation
#1
REVIEW
Jenna L Gollihue, Alexander G Rabchevsky
Mitochondrial dysfunction has been implicated in a multitude of diseases and pathological conditions- the organelles that are essential for life can also be major players in contributing to cell death and disease. Because mitochondria are so well established in our existence, being present in all cell types except for red blood cells and having the responsibility of providing most of our energy needs for survival, then dysfunctional mitochondria can elicit devastating cellular pathologies that can be widespread across the entire organism...
May 19, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28526334/regulatory-cis-and-trans-elements-of-mitochondrial-d-loop-driven-reporter-genes-in-budding-tunicates
#2
Kaz Kawamura, Yuhya Saitoh, Loriano Ballarin, Takeshi Sunanaga
To unveil the underlying mechanism of mitochondrial gene regulation associated with ageing and budding in the tunicate Polyandrocarpa misakiensis, mitochondrial non-coding-region (NCR)-containing reporter genes were constructed. PmNCR2.3K/GFP was expressed spatiotemporally in a pattern quite similar to mitochondrial 16SrRNA. The reporter gene expression was sensitive to high dose of rifampicin similar to mitochondrial genes, suggesting that the transcription indeed occurs in mitochondria. However, the gene expression also occurred in vivo in the cell nucleus and in vitro in the nuclear extracts...
May 16, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28502718/mitochondrial-nd5-mutation-mediated-elevated-ros-regulates-apoptotic-pathway-epigenetically-in-a-p53-dependent-manner-for-generating-pro-cancerous-phenotypes
#3
Rajnish Kumar Singh, Sunil Saini, Dileep Verma, Ponnusamy Kalaiarasan, Rameshwar N K Bamezai
We have previously observed concomitant events of mutations in mitochondrial and nuclear genes, along with elevated reactive oxygen species (ROS) and differential methylation within the promoters of nuclear genes in tumors and in vitro experiments of tumorigenesis. These observations have made it pertinent to replicate and understand the role of acquired mitochondrial condition in tuning a cell to accomplish a pro-cancerous state. Using a codon optimized vector system for exogenous over-expression and mitochondrial localization; we have characterized here the role of over-expressed wild type mtND5 and one of its non-synonymous somatic mutation, ND5:P265H...
May 11, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28499984/systemic-pten-akt1-mtor-pathway-activity-in-patients-with-normal-tension-glaucoma-and-ocular-hypertension-a-case-series
#4
Gerassimos Lascaratos, Kai-Yin Chau, Haogang Zhu, Despoina Gkotsi, Deborah Kamal, Ivan Gout, Philip J Luthert, Anthony H V Schapira, David F Garway-Heath
Glaucoma is the most common optic neuropathy in humans and the leading cause of irreversible blindness worldwide. Its prevalence and incidence increase exponentially with ageing and raised intraocular pressure (IOP), while increasing evidence suggests that systemic mitochondrial abnormalities may also be implicated in its pathogenesis. We have recently shown that patients who have not developed glaucoma despite being exposed for many years to high IOP (ocular hypertension - OHT) have more efficient mitochondria, measured in peripheral blood lymphocytes, when compared to age-similar controls and fast progressing normal tension glaucoma (NTG) patients...
May 9, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28499983/photostimulation-of-mitochondria-as-a-treatment-for-retinal-neurodegeneration
#5
Kathy Beirne, Malgorzata Rozanowska, Marcela Votruba
Absorption of photon energy by neuronal mitochondria leads to numerous downstream neuroprotective effects. Red and near infrared (NIR) light are associated with significantly less safety concerns than light of shorter wavelengths and they are therefore, the optimal choice for irradiating the retina. Potent neuroprotective effects have been demonstrated in various models of retinal damage, by red/NIR light, with limited data from human studies showing its ability to improve visual function. Improved neuronal mitochondrial function, increased blood flow to neural tissue, upregulation of cell survival mediators and restoration of normal microglial function have all been proposed as potential underlying mechanisms of red/NIR light...
May 9, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28499982/identification-of-fastkd2-compound-heterozygous-mutations-as-the-underlying-cause-of-autosomal-recessive-melas-like-syndrome
#6
Da Hye Yoo, Young-Chul Choi, Da Eun Nam, Sun Seong Choi, Ji Won Kim, Byung-Ok Choi, Ki Wha Chung
Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a condition that affects many parts of the body, particularly the brain and muscles. This study examined a Korean MELAS-like syndrome patient with seizure, stroke-like episode, and optic atrophy. Target sequencing of whole mtDNA and 73 nuclear genes identified compound heterozygous mutations p.R205X and p.L255P in the FASTKD2. Each of his unaffected parents has one of the two mutations, and both mutations were not found in 302 controls...
May 9, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28499981/mitochondrial-dysfunction-in-ocular-disease-focus-on-glaucoma
#7
Khalid Kamel, Michael Farrell, Colm O'Brien
Mitochondrial dysfunction commonly presents with ocular findings as a part of a systemic disorder. These ophthalmic manifestations can be the first sign of a mitochondrial abnormality, which highlights the key role of a comprehensive ophthalmic assessment. On the other hand, a number of visually disabling genetic and acquired eye diseases with no curative treatment show abnormal mitochondrial function. Recent advances in mitochondrial research have improved our understanding of previously unexplained ocular disorders utilising better diagnostic approaches...
May 9, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28478183/rna-editing-of-cytochrome-c-maturation-transcripts-is-responsive-to-the-energy-status-of-leaf-cells-in-arabidopsis-thaliana
#8
Yuzhe Sun, Yee-Song Law, Shifeng Cheng, Boon Leong Lim
Overexpression of AtPAP2, a phosphatase located on the outer membranes of chloroplasts and mitochondria, leads to higher energy outputs from these organelles. AtPAP2 interacts with seven MORF proteins of the editosome complex. RNA-sequencing analysis showed that the editing degrees of most sites did not differ significantly between OE and WT, except some sites on the transcripts of several cytochrome c maturation (Ccm) genes. Western blotting of 2D BN-PAGE showed that the patterns of CcmFN1 polypeptides were different between the lines...
May 3, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28476317/evolution-structure-and-membrane-association-of-ndufaf6-an-assembly-factor-for-nadh-ubiquinone-oxidoreductase-complex-i
#9
Bernard D Lemire
The NADH:ubiquinone oxidoreductase (complex I) is the largest member of the mitochondrial respiratory chain. Its FMN cofactor accepts two electrons from NADH and transfers them to ubiquinone via a chain of iron-sulphur centers. A central core of 14 highly conserved subunits can couple electron transfer to proton translocation. The mammalian enzyme has an additional ~30 accessory subunits. Complex I has important bioenergetic and metabolic functions and is a known source of reactive oxygen species; these functions link it to a number of hereditary and degenerative diseases...
May 2, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28438612/glucose-and-glutamine-in-the-mitochondrial-oxidative-metabolism-of-stem-cells
#10
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
#11
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/28396253/moderate-therapeutic-hypothermia-induces-multimodal-protective-effects-in-oxygen-glucose-deprivation-reperfusion-injured-cardiomyocytes
#12
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 8, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28396254/mutpred-mutational-load-analysis-shows-mildly-deleterious-mitochondrial-dna-variants-are-not-more-prevalent-in-alzheimer-s-patients-but-may-be-under-represented-in-healthy-older-individuals
#13
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/28365408/mitochondrial-dysfunction-underlying-outer-retinal-diseases
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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