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Mitochondrion

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https://www.readbyqxmd.com/read/28802668/two-novel-lncrnas-discovered-in-human-mitochondrial-dna-using-pacbio-full-length-transcriptome-data
#1
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
https://www.readbyqxmd.com/read/28802667/different-approaches-to-modeling-analysis-of-mitochondrial-swelling
#2
REVIEW
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 9, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28802666/humanin-directly-protects-cardiac-mitochondria-against-dysfunction-initiated-by-oxidative-stress-by-decreasing-complex-i-activity
#3
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
https://www.readbyqxmd.com/read/28802665/no-evidence-of-association-between-optic-neuritis-and-secondary-lhon-mtdna-mutations-in-patients-with-multiple-sclerosis
#4
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
https://www.readbyqxmd.com/read/28801230/a-mitochondrial-proteomics-view-of-complex-i-deficiency-in-candida-albicans
#5
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
https://www.readbyqxmd.com/read/28778567/unveiling-the-mystery-of-mitochondrial-dna-replication-in-yeasts
#6
REVIEW
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
https://www.readbyqxmd.com/read/28764911/cultural-diffusion-of-indo-aryan-languages-into-bangladesh-a-perspective-from-mitochondrial-dna
#7
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
https://www.readbyqxmd.com/read/28750948/mitochondrial-function-requires-ngly1
#8
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
https://www.readbyqxmd.com/read/28739512/modulation-of-mitochondrial-dysfunction-related-oxidative-stress-in-fibroblasts-of-patients-with-leigh-syndrome-by-inhibition-of-prooxidative-p66shc-pathway
#9
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
https://www.readbyqxmd.com/read/28729194/establishment-of-human-retinal-mitoscriptome-gene-expression-signature-for-diabetic-retinopathy-using-cadaver-eyes
#10
Gowthaman Govindarajan, Saumi Mathews, Karthik Srinivasan, Kim Ramasamy, Sundaresan Periyasamy
Diabetic retinopathy (DR) is a leading cause of blindness due to retinal microvasculature. We used microarray analysis for the first time to establish the retinal mitoscriptome gene expression signature for DR using human cadaver eyes. Among the 1042 genes, 60 (52-down, 8-up) and 39 (36-down, 3-up) genes were differentially expressed in the DR as compared to normal control and diabetic retinas respectively. These genes were mainly responsible for regulating angiogenesis, anti-oxidant defense mechanism, ATP production and apoptosis contributing to the disease pathology of DR...
July 17, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28729193/the-pattern-of-retinal-ganglion-cell-dysfunction-in-leber-hereditary-optic-neuropathy
#11
A Majander, A G Robson, C João, G E Holder, P F Chinnery, A T Moore, M Votruba, A Stockman, P Yu-Wai-Man
Leber inherited optic neuropathy (LHON) is characterized by subacute bilateral loss of central vision due to dysfunction and loss of retinal ganglion cells (RGCs). Comprehensive visual electrophysiological investigations (including pattern reversal visual evoked potentials, pattern electroretinography and the photopic negative response) performed on 13 patients with acute and chronic LHON indicate early impairment of RGC cell body function and severe axonal dysfunction. Temporal, spatial and chromatic psychophysical tests performed on 7 patients with acute LHON and 4 patients with chronic LHON suggest severe involvement or loss of the midget, parasol and bistratified RGCs associated with all three principal visual pathways...
July 17, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28716668/incomplete-penetrance-in-mitochondrial-optic-neuropathies
#12
Leonardo Caporali, Alessandra Maresca, Mariantonietta Capristo, Valentina Del Dotto, Francesca Tagliavini, Maria Lucia Valentino, Chiara La Morgia, Valerio Carelli
Incomplete penetrance characterizes the two most frequent inherited optic neuropathies, Leber's Hereditary Optic Neuropathy (LHON) and dominant optic atrophy (DOA), due to genetic errors in the mitochondrial DNA (mtDNA) and the nuclear DNA (nDNA), respectively. For LHON, compelling evidence has accumulated on the complex interplay of mtDNA haplogroups and environmental interacting factors, whereas the nDNA remains essentially non informative. However, a compensatory mechanism of activated mitochondrial biogenesis and increased mtDNA copy number, possibly driven by a permissive nDNA background, is documented in LHON; when successful it maintains unaffected the mutation carriers, but in some individuals it might be hampered by tobacco smoking or other environmental factors, resulting in disease onset...
July 14, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28716667/pupillometric-evaluation-of-the-melanopsin-containing-retinal-ganglion-cells-in-mitochondrial-and-non-mitochondrial-optic-neuropathies
#13
Shakoor Ba-Ali, Henrik Lund-Andersen
In recent years, chromatic pupillometry is used in humans to evaluate the activity of melanopsin expressing intrinsic photosensitive retinal ganglion cells (ipRGCs). Blue light is used to stimulate the ipRGCs and red light activates the rod/cone photoreceptors. The late re-dilation phase of pupillary light reflex is primarily driven by the ipRGCs. Optic neuropathies i.e. Leber hereditary optic neuropathy (LHON), autosomal dominant optic atrophy (ADOA), nonarteritic anterior ischemic optic neuropathy (NAION), glaucoma, optic neuritis and idiopathic intracranial hypertension (IIH) are among the diseases, which have been subject to pupillometric studies...
July 14, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28716666/mouse-cardiac-mitochondria-do-not-separate-in-subsarcolemmal-and-interfibrillar-subpopulations
#14
Ulrike B Hendgen-Cotta, Sonja Esfeld, Holger Jastrow, Matthias Totzeck, Joachim Altschmied, Christine Goy, Judith Haendeler, Elke Winterhager, Tienush Rassaf
Cardiomyocytes consist of longitudinally oriented myofibril bundles with a misaligned composition caused by the uneven contours of the intercalated discs. The cytoplasmic space harbors the organelles, including mitochondria. This study investigated whether cardiomyocytes contain spatially and ultrastructurally discrete pools of mitochondria that can be separated for structurally and functionally appraisal in (patho)physiology. Transmission electron microscopy disclosed continuous transitions of mitochondria without attributable characteristics from beneath the sarcolemma directly into the barrier-free cytoplasmic space between myofibrils...
July 14, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28694194/lyrm7-associated-complex-iii-deficiency-a-clinical-molecular-genetic-mr-tomographic-and-biochemical-study
#15
Maja Hempel, Laura S Kremer, Konstantinos Tsiakas, Bader Alhaddad, Tobias B Haack, Ulrike Löbel, René G Feichtinger, Wolfgang Sperl, Holger Prokisch, Johannes A Mayr, René Santer
LYRM7 is involved in the last steps of mitochondrial complex III assembly where it acts as a chaperone for the Rieske iron‑sulfur (Fe-S) protein in the mitochondrial matrix. Using exome sequencing, we identified homozygosity for a splice site destroying 4 base pair deletion in LYRM7 in a child with recurrent lactic acidotic crises and distinct early-onset leukencephalopathy. Sanger sequencing showed variant segregation in similarly affected family members. Functional analyses revealed a reduced amount of the Rieske Fe-S protein, which was restored after re-expression of LYRM7...
July 7, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28687512/recessive-mutation-in-exosc3-associates-with-mitochondrial-dysfunction-and-pontocerebellar-hypoplasia
#16
Gudrun Schottmann, Sylvie Picker-Minh, Jana Marie Schwarz, Esther Gill, Richard J T Rodenburg, Werner Stenzel, Angela M Kaindl, Markus Schuelke
Recessive mutations in EXOSC3, encoding a subunit of the human RNA exosome complex, cause pontocerebellar hypoplasia type 1b (PCH1B). We report a boy with severe muscular hypotonia, psychomotor retardation, progressive microcephaly, and cerebellar atrophy. Biochemical abnormalities comprised mitochondrial complex I and pyruvate dehydrogenase complex (PDHc) deficiency. Whole exome sequencing uncovered a known EXOSC3 mutation p.(D132A) as the underlying cause. In patient fibroblasts, a large portion of the EXOSC3 protein was trapped in the cytosol...
July 4, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28673863/mpv17-hepatocerebral-mitochondrial-dna-depletion-syndrome-presenting-as-acute-flaccid-paralysis-a-case-report
#17
Anjan Pyal, Arumugam Paramasivam, Angamuthu Kannan Meena, Velpula Bhagya Bhavana, Kumarasamy Thangaraj
Mutations in human MPV17 have been reported in patients with severe mitochondrial DNA (mtDNA) depletion manifesting as early childhood onset failure to thrive, hypoglycemia, encephalopathy and progressive liver failure. We describe an 11 year old girl, born to consanguineous parents, who presented with rapidly progressive weakness of all 4 limbs with symmetrical proximal and distal weakness, gastrointestinal disease and leukoencephalopathy. Genetic analysis of the patient revealed a homozygous pathogenic mutation c...
June 30, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28669809/functional-nanosome-for-enhanced-mitochondria-targeted-gene-delivery-and-expression
#18
Yoonhee Bae, Min Kyo Jung, Su Jeong Song, Eric S Green, Seulgi Lee, Hyun-Sook Park, Seung Hun Jeong, Jin Han, Ji Young Mun, Kyung Soo Ko, Joon Sig Choi
Mitochondria dysfunction plays a role in many human diseases. Therapeutic techniques for these disorders require novel delivery systems that can specifically target and penetrate mitochondria. In this study, we report a novel nanosome composed of dequalinium-DOTAP-DOPE (1,2 dioleoyl-3-trimethylammonium-propane-1,2-dioleoyl-sn-glycero-3-phosphoethanolamine) (DQA80s) as a potential mitochondria-targeting delivery vector. The functional DQAsome, DQA80s, showed enhanced transfection efficiency compared to a vector DQAsomes in HeLa cells and dermal fibroblasts...
June 29, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28668667/factors-influencing-codon-usage-of-mitochondrial-nd1-gene-in-pisces-aves-and-mammals
#19
Arif Uddin, Monisha Nath Choudhury, Supriyo Chakraborty
Animal mitochondrial genome harbours 13 protein coding genes which regulate the process of respiration. The mitochondrial NADH dehydrogenase 1 (MT-ND1) gene, one of the 13 protein-coding genes, encodes the NADH dehydrogenase 1 enzyme of the respiratory chain. Analysis of codon usage bias (CUB) acquires importance for better understanding of the molecular biology, new gene discovery, design of transgenes and gene evolution. The MT-ND1 gene seems to be a good candidate for analyzing codon usage pattern, since no work has yet been reported...
June 28, 2017: Mitochondrion
https://www.readbyqxmd.com/read/28655663/resistive-flow-sensing-of-vital-mitochondria-with-nanoelectrodes
#20
Katayoun Zand, Ted D A Pham, Jinfeng Li, Weiwei Zhou, Douglas C Wallace, Peter J Burke
We report label-free detection of single mitochondria with high sensitivity using nanoelectrodes. Measurements of the conductance of carbon nanotube transistors show discrete changes of conductance as individual mitochondria flow over the nanoelectrodes in a microfluidic channel. Altering the bioenergetic state of the mitochondria by adding metabolites to the flow buffer induces changes in the mitochondrial membrane potential detected by the nanoelectrodes. During the time when mitochondria are transiently passing over the nanoelectrodes, this (nano) technology is sensitive to fluctuations of the mitochondrial membrane potential with a resolution of 10mV with temporal resolution of order milliseconds...
June 24, 2017: Mitochondrion
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