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Mitochondrion

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https://www.readbyqxmd.com/read/28634151/speech-and-swallowing-abnormalities-in-adults-with-polg-associated-ataxia-polg-a
#1
Adam P Vogel, Natalie Rommel, Andreas Oettinger, Marius Horger, Patrick Krumm, Eva-Maria Kraus, Ludger Schöls, Matthis Synofzik
BACKGROUND: Mutations in the nuclear-encoded mitochondrial DNA polymerase gamma (POLG) can result in a wide spectrum of neurological deficits. A common presentation is progressive ataxia (POLG-A) which includes impaired speech and swallowing. The nature, severity and impact of these deficits in POLG-A is not known. A comprehensive quantitative and qualitative characterization of dysarthria and dysphagia in this recurrent ataxia disorder will assist in diagnostics, provide insights into the underlying pathology, and establish the foundation for future therapy trials...
June 17, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28603048/mitonuclear-epistasis-and-mitochondrial-disease
#2
LETTER
Edward H Morrow, M Florencia Camus
No abstract text is available yet for this article.
June 7, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28579530/late-onset-nonsyndromic-hearing-loss-in-a-dongxiang-chinese-family-is-associated-with-the-593t-c-variant-in-the-mitochondrial-trna-phe-gene
#3
Xiaowan Chen, Zhipeng Nie, Fang Wang, Jianchao Wang, Xiao-Wen Liu, Jing Zheng, Yu-Fen Guo, Min-Xin Guan
We report here the clinical, genetic, molecular and biochemical characterization of a four-generation Dongxiang Chinese pedigree with suggestively maternally transmitted non-syndromic hearing loss. Five of 10 matrilineal relatives exhibited variable severity and age at onset of sensorineural hearing loss. The average ages at onset of hearing loss in matrilineal relatives of this family were 29years. Molecular analysis of their mitochondrial genomes identified the tRNA(Phe) 593T>C variant belonging to Asian haplogroup G2a2a...
June 1, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28576558/metabolomics-of-mitochondrial-disease
#4
REVIEW
Karien Esterhuizen, Francois H van der Westhuizen, Roan Louw
Mitochondrial disease (MD) diagnostics and disease progression investigations have traditionally relied very little on metabolic data, due to a lack of biomarker sensitivity and specificity. The recent drive to find novel, low intervention biomarkers and new therapeutic approaches have revived an interest in what metabolic data can offer, as presented in this timely review. We review how metabolomics has been applied to MD and provide an extensive overview of the reported metabolic perturbations and common mechanistic features that may provide a basis for future research...
May 30, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28572055/estrogen-regulates-spatially-distinct-cardiac-mitochondrial-subpopulations
#5
Rogério Faustino Ribeiro Junior, Paula Lopes Rodrigues, Elis Aguiar Morra, Karoline Sousa Ronconi, Patrícia Ribeiro Do Val Lima, Marcella Leite Porto, Maylla Ronacher Simões, Dalton Valentim Vassallo, Suely Gomes Figueiredo, Ivanita Stefanon
Increased susceptibility to permeability transition pore (mPTP) is a significant concern to decreased cardiac performance in postmenopausal females. The goal of this study was to assess the effects of estrogen deficiency on the two spatially distinct mitochondrial subpopulations from left ventricle: subsarcolemmal mitochondria (SSM) and intermyofibrillar mitochondria (IFM) based on: morphology, membrane potential, oxidative phosphorylation, mPTP and reactive oxygen species production. Female rats (8weeks old) that underwent bilateral ovariectomy were randomly assigned to receive daily treatment with placebo (OVX), estrogen replacement (OVX+E2) and Sham for 60days...
May 29, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28552593/tyrphostin-a9-improves-blastocyst-development-in-porcine-embryos-through-induction-of-dynamin-related-protein-1-dependent-mitochondrial-fission
#6
Jae-Hyun Ahn, Hyo-Jin Park, Jin-Woo Kim, Jae-Young Park, Soo-Yong Park, Seul-Gi Yang, Chang-Hyun Kim, Seung-Bin Yoon, Sun-Uk Kim, Kyu-Tae Chang, Deog-Bon Koo
Mitochondrial dynamics are associated with the development of porcine embryos. However, little is known about the effects of mitochondrial dynamics-related genes (Drp1 and pDrp1-Ser616) on early porcine embryo development. Here, we investigated the effect of Drp1-dependent mitochondrial fission signaling on the development of porcine embryos using the mitochondrial fission inducer, tyrphostin A9 (TA9). We determined that TA9 (1μM) treated embryos were increased the mitochondrial functions, blastocyst development rate and quality, as well as decreased mitochondria-specific superoxide and mitochondrial apoptosis...
May 26, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28549843/topical-coenzyme-q10-demonstrates-mitochondrial-mediated-neuroprotection-in-a-rodent-model-of-ocular-hypertension
#7
Benjamin Michael Davis, Kailin Tian, Milena Pahlitzsch, Jonathan Brenton, Nivedita Ravindran, Gibran Butt, Giulia Malaguarnera, Eduardo M Normando, Li Guo, M Francesca Cordeiro
Coenzyme Q10 (CoQ10) is a mitochondrial-targeted antioxidant with known neuroprotective activity. Its ocular effects when co-solubilised with α-tocopherol polyethylene glycol succinate (TPGS) were evaluated. In vitro studies confirmed that CoQ10 was significantly protective in different retinal ganglion cell (RGC) models. In vivo studies in Adult Dark Agouti (DA) rats with unilateral surgically-induced ocular hypertension (OHT) treated with either CoQ10/TPGS micelles or TPGS vehicle twice daily for three weeks were performed, following which retinal cell health was assessed in vivo using DARC (Detection of Apoptotic Retinal Cells) and post-mortem with Brn3a histological assessment on whole retinal mounts...
May 24, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28549842/mitochondrial-dysfunction-and-oxidative-stress-in-corneal-disease
#8
Neeru A Vallabh, Vito Romano, Colin E Willoughby
The cornea is the anterior transparent surface and the main refracting structure of the eye. Mitochondrial dysfunction and oxidative stress are implicated in the pathogenesis of inherited (e.g. Kearns Sayre Syndrome) and acquired corneal diseases (e.g. keratoconus and Fuchs endothelial corneal dystrophy). Both antioxidants and reactive oxygen species are found in the healthy cornea. There is increasing evidence of imbalance in the oxidative balance and mitochondrial function in the cornea in disease states...
May 23, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28533168/prospects-for-therapeutic-mitochondrial-transplantation
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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