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Tina Pawar, Magnar Bjørås, Arne Klungland, Lars Eide
The mitochondrial DNA (mtDNA) resides in the vicinity of energy-rich reactions. Thus, chemical modifications of mtDNA might mirror mitochondrial processes and could serve as biomarkers of metabolic processes in the mitochondria. This hypothesis was tested by assessing modifications at 17 different sites in the mtDNA as a function of cell type, oxidative stress and mitochondrial activity. Two mouse mutants with a metabolic phenotype were compared to wild-type (WT) mice: the ogg1(-/-) mouse that lacks the 8-oxoguanine DNA glycosylase (OGG1), and the alkbh7(-/-) mouse missing the ALKBH7 protein that has been implicated in fatty acid oxidation...
September 8, 2017: Mitochondrion
D Lattuada, G Alfonsi, L Roncati, T Pusiol, A Bulfoni, S Ferrero, A M Lavezzi
In literature there are no data related to mitochondrial DNA (mtDNA) content in sudden intrauterine unexplained death syndrome (SIUDS). To test the hypothesis that a quantitative defect of mtDNA would play a role in the pathogenesis of SIUDS, mtDNA content was measured in cerebral cortex of 9 SIUDS and in 7 controls. The median (interquartile range) mtDNA in SIUDS and controls was 14,000 (8600-33,500), 3400 (0-8500) copies per nuclear DNA, respectively (p=0.007). Mitochondria are involved in SIUDS and higher mitochondrial DNA content may be a biomarker of this syndrome...
September 7, 2017: Mitochondrion
Yoko A Ito, Adriana Di Polo
Retinal ganglion cells, the neurons that selectively die in glaucoma and other optic neuropathies, are endowed with an exceedingly active metabolism and display a particular vulnerability to mitochondrial dysfunction. Mitochondria are exquisitely dynamic organelles that are continually responding to endogenous and environmental cues to readily meet the energy demand of neuronal networks. The highly orchestrated regulation of mitochondrial biogenesis, fusion, fission, transport and degradation is paramount for the maintenance of energy-expensive synapses at RGC dendrites and axon terminals geared for optimal neurotransmission...
September 1, 2017: Mitochondrion
Abhijit Babaji Shinde, Ritesh Kumar Baboota, Simone Denis, Ursula Loizides-Mangold, Annelies Peeters, Marc Espeel, Ana Rita Malheiro, Howard Riezman, Stefan Vinckier, Frédéric M Vaz, Pedro Brites, Sacha Ferdinandusse, Paul P Van Veldhoven, Myriam Baes
The structural disruption of the mitochondrial inner membrane in hepatocytes lacking functional peroxisomes along with selective impairment of respiratory complexes and depletion of mitochondrial DNA was previously reported. In search for the molecular origin of these mitochondrial alterations, we here show that these are tissue selective as they do neither occur in peroxisome deficient brain nor in peroxisome deficient striated muscle. Given the hepatocyte selectivity, we investigated the potential involvement of metabolites that are primarily handled by hepatic peroxisomes...
August 30, 2017: Mitochondrion
Relu Cocoş, Sorina Schipor, Corin Badiu, Florina Raicu
We aimed to analyze the contribution of mitochondrial DNA (mtDNA) haplogroups of the mtDNA control region to thyroid cancer risk in a population from southeastern Europe consisting of 235 thyroid tumor patients, including 114 patients with thyroid follicular adenoma, 121 patients with papillary thyroid carcinoma, and 419 healthy controls. Binary logistic regression with adjustment for age and gender revealed that mtDNA haplogroup K was significantly associated with a protective role for thyroid cancer in the combined tumor group versus controls...
August 26, 2017: Mitochondrion
Aziza A A Adam, Martien van Wenum, Vincent A van der Mark, Aldo Jongejan, Perry D Moerland, Riekelt H Houtkooper, Ronald J A Wanders, Ronald P Oude Elferink, Robert A F M Chamuleau, Ruurdtje Hoekstra
BACKGROUND: Human liver cell lines, like HepaRG and C3A, acquire higher functionality when cultured in the AMC-Bio-Artificial Liver (AMC-BAL). The three main differences between BAL and monolayer culture are the oxygenation (40% vs 20%O2), dynamic vs absent medium perfusion and 3D vs 2D configuration. Here, we investigated the background of the differences between BAL-cultures and monolayers. METHODS: We performed whole-genome microarray analysis on HepaRG monolayer and BAL-cultures...
August 24, 2017: Mitochondrion
Prashant Bajpai, Aarti Darra, Anurag Agrawal
Human mitochondria are descendants of microbes and altered mitochondrial function has been implicated in processes ranging from ageing to diabetes. Recent work has highlighted the importance of gut microbial communities in human health and disease. While the spotlight has been on the influence of such communities on the human immune system and the extraction of calories from otherwise indigestible food, an important but less investigated link between the microbes and mitochondria remains unexplored. Microbial metabolites including short chain fatty acids as well as other molecules such as pyrroloquinoline quinone, fermentation gases, and modified fatty acids influence mitochondrial function...
August 24, 2017: Mitochondrion
M Kolko
No abstract text is available yet for this article.
August 22, 2017: Mitochondrion
Anouk Tosserams, Constantinos Papadopoulos, Claude Jardel, Isabelle Lemière, Norma B Romero, Pascale De Lonlay, Karim Wahbi, Nicol Voermans, Jean-Yves Hogrel, Pascal Laforêt
We report the clinical, morphological and molecular features of two patients with autosomal recessive SLC25A4 (ANT1) gene mutations. Furthermore, all previously published cases are reviewed to identify valuable features for future diagnosis. Patients present a common phenotype with exercise intolerance, hyperlactatemia, and hypertrophic cardiomyopathy. Muscle biopsies show wide sub-sarcolemmal mitochondrial aggregates, and increased activities of all respiratory chain complexes. The phenotype of recessive SLC25A4 (ANT1) mutations although rare, is homogenous and easily recognizable and could help orientate the molecular analysis in adults with exercise intolerance associated with hyperlactatemia...
August 17, 2017: Mitochondrion
Anna L Guyatt, Kimberley Burrows, Philip A I Guthrie, Sue Ring, Wendy McArdle, Ian N M Day, Raimondo Ascione, Debbie A Lawlor, Tom R Gaunt, Santiago Rodriguez
The mitochondrial genome is present at variable copy number between individuals. Mitochondria are vulnerable to oxidative stress, and their dysfunction may be associated with cardiovascular disease. The association of mitochondrial DNA copy number with cardiometabolic risk factors (lipids, glycaemic traits, inflammatory markers, anthropometry and blood pressure) was assessed in two independent cohorts of European origin women, one in whom outcomes were measured at mean (SD) age 30 (4.3) years (N=2278) and the second at 69...
August 15, 2017: Mitochondrion
Shreya Ahana Ayyub, Aswathy S L, Divya Dobriyal, Srinivas Aluri, Linda L Spremulli, Umesh Varshney
Initiation factor 3 (IF3) is a conserved translation factor. Mutations in mitochondrial IF3 (IF3mt) have been implicated in disease pathology. Escherichia coli infCΔ55, compromised for IF3 activity, has provided an excellent heterologous system for IF3mt structure-function analysis. IF3mt allowed promiscuous initiation from AUA, AUU and ACG codons but avoided initiation with initiator tRNAs lacking the conserved 3GC pairs in their anticodon stems. Expression of IF3mt N-terminal domain, or IF3mt devoid of its typical N-, and C-terminal extensions improved fidelity of initiation in E...
August 10, 2017: Mitochondrion
Sabzali Javadov, Xavier Chapa-Dubocq, Vladimir Makarov
Mitochondria are critical players involved in both cell life and death through multiple pathways. Structural integrity, metabolism and function of mitochondria are regulated by matrix volume due to physiological changes of ion homeostasis in cellular cytoplasm and mitochondria. Ca(2+) and K(+) presumably play a critical role in physiological and pathological swelling of mitochondria when increased uptake (influx)/decreased release (efflux) of these ions enhances osmotic pressure accompanied by high water accumulation in the matrix...
August 10, 2017: Mitochondrion
Shan Gao, Xiaoxuan Tian, Hong Chang, Yu Sun, Zhenfeng Wu, Zhi Cheng, Pengzhi Dong, Qiang Zhao, Jishou Ruan, Wenjun Bu
In this study, we introduced a general framework to use PacBio full-length transcriptome sequencing for the investigation of mitochondrial RNAs. As a result, we produced the first full-length human mitochondrial transcriptome based on the public PacBio data and characterized the human mitochondrial transcriptome with more comprehensive and accurate information. Other results included the determination of the H-strand primary transcript, the identification of ND5/ND6AS/tRNA(Glu)AS, the discovery of palindrome small RNAs and the construction of "cleavage" model...
August 9, 2017: Mitochondrion
Savitree Thummasorn, Krekwit Shinlapawittayatorn, Juthamas Khamseekaew, Thidarat Jaiwongkam, Siriporn C Chattipakorn, Nipon Chattipakorn
Humanin (HN) is an endogenous peptide that exerts cytoprotection against oxidative stress and apoptosis. We recently reported that Humanin analogue (HNG) pretreatment can reduce reactive oxygen species production in the heart subjected to ischemia/reperfusion (I/R) injury via attenuating mitochondrial dysfunction. However, it is unclear if HNG has direct effects on mitochondrial function against oxidative stress. Thus, we sought to determine the effects of HNG on mitochondrial function under hydrogen peroxide (H2O2) induced oxidative stress in isolated cardiac mitochondria...
August 9, 2017: Mitochondrion
Sasan Andalib, Mahnaz Talebi, Ebrahim Sakhinia, Mehdi Farhoudi, Homayoun Sadeghi-Bazargani, Nooshin Masoodian, Manouchehr Seyedi Vafaee, Albert Gjedde
Leber's Hereditary Optic Neuropathy (LHON) shares features with Multiple Sclerosis (MS). Both diseases develop optic lesions. Frequent secondary LHON mutations in MS patients may explain the optic damage. Here, we tested the hypothesis that secondary LHON mutations are associated with optic neuritis (ON) in MS patients. We recruited 56 MS subjects with ON and 47 MS subjects without ON. DNA was extracted by salting out, after sampling of peripheral blood from each participant. We completed Polymerase Chain Reaction-Restriction Fragment Length Polymorphism (PCR-RFLP) analysis with appropriate primers and restriction endonucleases for seven secondary LHON mutations...
August 9, 2017: Mitochondrion
Xiaodong She, Pengyi Zhang, Ying Gao, Lulu Zhang, Qiong Wang, Hui Chen, Richard Calderone, Weida Liu, Dongmei Li
Proteomic analyses were carried out on isolated mitochondrial samples of C. albicans from gene-deleted mutants (nuo1Δ, nuo2Δ and goa1Δ) as well as the parental strain in order to better understand the contribution of these three fungal-specific mitochondrial ETC complex I (CI) subunits to cellular activities. Herein, we identify 2333 putative proteins from four strains, in which a total of 663 proteins (28.5%) are putatively located in mitochondria. Comparison of protein abundances between mutants and the parental strain reveal 146 differentially-expressed proteins, of which 78 are decreased and 68 are increased in at least one mutant...
August 8, 2017: Mitochondrion
Xin Jie Chen, George Desmond Clark-Walker
Conventional DNA replication is initiated from specific origins and requires the synthesis of RNA primers for both the leading and lagging strands. In contrast, the replication of yeast mitochondrial DNA is origin-independent. The replication of the leading strand is likely primed by recombinational structures and proceeded by a rolling circle mechanism. The coexistent linear and circular DNA conformers facilitate the recombination-based initiation. The replication of the lagging strand is poorly understood...
August 1, 2017: Mitochondrion
Yu-Chun Li, Hua-Wei Wang, Jiao-Yang Tian, Rui-Lei Li, Zia Ur Rahman, Qing-Peng Kong
Although both linguistic and historical studies indicated only a small group of Aryans had been involved into the diffusion of Indo-Aryan languages into Bangladesh, no genetic studies had been carried out to prove this notion. By studying mitochondrial DNA variants of 240 Bengali speakers in Bangladesh, among which 23 mitogenomes are completely sequenced, we found a high proportion of South Asian components in this group. By contrast, only a small proportion of lineages can be traced back to western Eurasia, which could be attributed to recent gene flow...
July 29, 2017: Mitochondrion
Jianping Kong, Min Peng, Julian Ostrovsky, Young Joon Kwon, Olga Oretsky, Elizabeth M McCormick, Miao He, Yair Argon, Marni J Falk
Mitochondrial respiratory chain (RC) diseases and congenital disorders of glycosylation (CDG) share extensive clinical overlap but are considered to have distinct cellular pathophysiology. Here, we demonstrate that an essential physiologic connection exists between cellular N-linked deglycosylation capacity and mitochondrial function. Following identification of altered muscle and liver mitochondrial amount and function in two children with a CDG subtype caused by NGLY1 deficiency, we evaluated mitochondrial physiology in NGLY1 disease human fibroblasts, and in NGLY1-knockout mouse embryonic fibroblasts and C...
July 25, 2017: Mitochondrion
Aleksandra Wojtala, Agnieszka Karkucinska-Wieckowska, Vilma A Sardao, Joanna Szczepanowska, Pawel Kowalski, Maciej Pronicki, Jerzy Duszynski, Mariusz R Wieckowski
The mitochondrial respiratory chain, and in particular, complex I, is a major source of reactive oxygen species (ROS) in cells. Elevated levels of ROS are associated with an imbalance between the rate of ROS formation and the capacity of the antioxidant defense system. Increased ROS production may lead to oxidation of DNA, lipids and proteins and thus can affect fundamental cellular processes. The aim of this study was to investigate the magnitude of intracellular oxidative stress in fibroblasts of patients with Leigh syndrome with defined mutations in complex I...
July 21, 2017: Mitochondrion
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