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Mai Tsujimura, Takakazu Kaneko, Tomoaki Sakamoto, Seisuke Kimura, Masayoshi Shigyo, Hiroshi Yamagishi, Toru Terachi
The structures of plant mitochondrial genomes are more complex than those of animals. One of the reasons for this is that plant mitochondrial genomes typically have many long and short repeated sequences and intra- and intermolecular recombination may create various DNA molecules in this organelle. Recombination may sometimes create a novel gene that causes cytoplasmic male sterility (CMS). The onion has several cytoplasm types, with some causing CMS while others do not. The complete mitochondrial genome sequence of the onion was reported for an inbred line with CMS-S cytoplasm; however, the number of differences between onion strains remains unclear, and studies on purified mitochondrial DNA (mtDNA) have not yet been performed...
July 10, 2018: Mitochondrion
Kalliopi Sofou, Pashtun Shahim, Már Tulinius, Kaj Blennow, Henrik Zetterberg, Niklas Mattsson, Niklas Darin
We studied the biomarker patterns related to axonal injury, astrogliosis and amyloid metabolism in cerebrospinal fluid (CSF) of children and adolescents with mitochondrial encephalopathy and identified correlations with phenotype and survival outcome. Forty-six pediatric patients with genetically verified mitochondrial encephalopathy and twenty-two controls investigated at the Queen Silvia Children's Hospital, Sweden, were included. CSF lactate and neurofilament light (NF-L) were significantly increased in patients with mitochondrial encephalopathy compared to controls...
July 9, 2018: Mitochondrion
Amel Karaa, Ibrahim Elsharkawi, Mark A Clapp, Cristy Balcells
A retrospective survey assessed the gynecologic, obstetric and fertility history of 103 women with mitochondrial disease (MD)/dysfunction (Md) aged 16 to 75 who had previously been pregnant. Most participants (34%) had a mitochondrial myopathy and there were 248 combined pregnancies with live deliveries (average 3.6 pregnancies/woman). In general, pregnancy in those with MD/Md appears to exacerbate some constitutional and neurological symptoms and may be more frequently associated with common obstetric complications, but this did not appear to result in worse pregnancy outcomes...
July 7, 2018: Mitochondrion
Sanjiban Chakrabarty, Shama Prasada Kabekkodu, Rana P Singh, K Thangaraj, Keshav K Singh, Kapaettu Satyamoorthy
Mitochondrial biology has become an area of intense research owing to the unique physiology of the organelle and its role in several types of cancers and other disorders. It has been found that mitochondria-encoded proteins, mitochondrial DNA and even RNA influence the functioning of the cell in more ways than were previously imagined. This may contribute to disease phenotypes which require detailed investigation and communication to the community health care providers. Additionally, this provides several novel avenues in drug designing against various cancers, neurodegenerative diseases and other metabolic disorders...
June 23, 2018: Mitochondrion
Arshia Angural, Indu Sharma, Pranav Pandoh, Varun Sharma, Akshi Spolia, Ekta Rai, Vinod Singh, Sushil Razdan, Kamal Kishore Pandita, Swarkar Sharma
Leigh Syndrome (LS) is a rare, hereditary progressive neurodegenerative disorder of infancy or early childhood associated with a highly variable clinical presentation even among siblings. Further, genetic heterogeneity makes its diagnosis complicated. Its causative genetic variations are notified in some of the mitochondrial and nuclear genes. Here, we report an atypical case of LS in a 9-year-old boy associated with a novel variation in MT-ATP6 gene. The atypical findings were Bilateral Basal Ganglia Calcification (BGC) and late survival age in the patient...
June 18, 2018: Mitochondrion
Xuhua Xia
There is a long-held belief that a mutation gradient exists along vertebrate mtDNA, mediated by mitochondrial replication that leaves different parts of the H-strand exposed in single-stranded state for different durations (DssH ). However, the predicted mutation gradient and its tests suffer from both conceptual and empirical problems. I assembled representative mammalian, avian and crocodilian mtDNA to test this prediction. I measured substitution rates at codon positions 1 and 2 (S12 ) and at codon position 3 (S3 ), as well as synonymous and nonsynonymous substitution rates, and checked their change along the hypothetical gradient...
June 14, 2018: Mitochondrion
Kunal Kumar, Akash Sabarwal, Rana P Singh
Mancozeb (Manganese ethylene bis-dithiocarbamate with zinc salt) is a dithiocarbamate fungicide used to control fungal disease in many fruit plants, flowers and the maintenance of field crops. The effect of mancozeb on cell viability of human gastric adenocarcinoma AGS, SNU-1 cells and human normal FHs 74 Int cells were investigated. This study demonstrated that mancozeb was able to inhibit cell proliferation by 56-82% at 5-10 μM concentrations after 48 h. Mancozeb treatment for 48 h resulted in 33% (P < 0...
June 11, 2018: Mitochondrion
M Aravintha Siva, R Mahalakshmi, Dipita Bhakta-Guha, Gunjan Guha
In the recent years, the reported cases of mitochondrial disorders have reached a colossal number. These disorders spawn a sundry of pathological conditions, which lead to pernicious symptoms and even fatality. Due to the unpredictable etiologies, mitochondrial diseases are putatively referred to as "mystondria" (mysterious diseases of mitochondria). Although present-day research has greatly improved our understanding of mitochondrial disorders, effective therapeutic interventions are still at the precursory stage...
June 8, 2018: Mitochondrion
Martine Uittenbogaard, Christine A Brantner, ZiShui Fang, Lee-Jun Wong, Andrea Gropman, Anne Chiaramello
Little is known about the molecular mechanism of the rare coexistence of Leber's Hereditary Optic Neuropathy (LHON) and multiple sclerosis (MS), also known as the Harding's syndrome. In this study, we provide novel evidence that the m.11778A > G variant causes a defective metabolic interplay between mitochondrial oxidative phosphorylation and glycolysis. We used dermal fibroblasts derived from a female proband exhibiting clinical symptoms compatible with LHON-MS due to the presence of the pathogenic m...
June 8, 2018: Mitochondrion
Rebecca Hanna, Jonathan M Crowther, Pallav A Bulsara, Xuying Wang, David J Moore, Mark A Birch-Machin
Intrinsic and extrinsic factors that induce cellular oxidative stress damage tissue integrity and promote ageing, resulting in accumulative strand breaks to the mitochondrial DNA (mtDNA) genome. Limited repair mechanisms and close proximity to superoxide generation make mtDNA a prominent biomarker of oxidative damage. Using human DNA we describe an optimised long-range qPCR methodology that sensitively detects mtDNA strand breaks relative to a suite of short mitochondrial and nuclear DNA housekeeping amplicons, which control for any variation in mtDNA copy number...
May 4, 2018: Mitochondrion
Vitaly D Samuilov, Dmitry B Kiselevsky, Alexander V Oleskin
This work focuses on the effect of mitochondria-targeted quinones (SkQs) on plants. SkQs with antioxidant properties are accumulated in the mitochondria of pea cells and suppress the generation of reactive oxygen species. At nanomolar concentrations, SkQs prevented the death of pea leaf epidermal or guard cells caused by chitosan, bacterial lipopolysaccharide or KCN. The protective effect of SkQs was removed by a protonophoric uncoupler. SkQs at micromolar concentrations inhibited the O2 evolution by illuminated chloroplasts and stimulated the respiration of mitochondria...
May 1, 2018: Mitochondrion
Deepa R Avisetti, Niharika Amireddy, Shasi V Kalivendi
Amongst various therapeutic properties of the natural product embelin, its anti-cancer effects are being extensively studied. We observed that, embelin induced apoptosis in A549 cells lacking functional mitochondria (ρ0 cells) indicating that its mitochondrial effects are not primarily responsible for its anti-cancer activity. However, p38 mediated activation of p53 was found to play a pivotal role in governing the apoptotic activity of embelin due to the following observations: a time-dependent activation of p53 and apoptosis by embelin; selective inhibition of p38 inhibited embelin-induced p53 levels...
April 30, 2018: Mitochondrion
Daniel Jablonski, Václav Gvoždík, Lukáš Choleva, David Jandzik, Jiří Moravec, Zdeněk Mačát, Milan Veselý
The maternal origin of isolated populations of the common wall lizard (Podracis muralis) in the Czech Republic, representing the north-eastern range border of the species, was addressed. We compared mitochondrial DNA sequences of the cytochrome b gene of samples from these populations with those from within the continuous range in Slovakia, the northern Balkan region, and those available from previous studies. We recorded five main haplogroups in the studied region, with all available Central European samples belonging to the same haplogroup...
April 22, 2018: Mitochondrion
A Phillip West
No abstract text is available yet for this article.
July 2018: Mitochondrion
Seong-Jun Kim, Dae-Gyun Ahn, Gulam H Syed, Aleem Siddiqui
Viruses alter cellular physiology and function to establish cellular environment conducive for viral proliferation. Viral immune evasion is an essential aspect of viral persistence and proliferation. The multifaceted mitochondria play a central role in many cellular events such as metabolism, bioenergetics, cell death, and innate immune signaling. Recent findings accentuate that viruses regulate mitochondrial function and dynamics to facilitate viral proliferation. In this review, we will discuss how viruses exploit mitochondrial dynamics to modulate mitochondria-mediated antiviral innate immune response during infection...
July 2018: Mitochondrion
Charles S Dela Cruz, Min-Jong Kang
Inflammation represents a comprehensive host response to external stimuli for the purpose of eliminating the offending agent, minimizing injury to host tissues and fostering repair of damaged tissues back to homeostatic levels. In normal physiologic context, inflammatory response culminates with the resolution of infection and tissue damage response. However, in a pathologic context, persistent or inappropriately regulated inflammation occurs that can lead to chronic inflammatory diseases. Recent scientific advances have integrated the role of innate immune response to be an important arm of the inflammatory process...
July 2018: Mitochondrion
Hector Sandoval, Srikanth Kodali, Jin Wang
B cells are responsible for protective antibody production after differentiation into antibody-secreting cells during humoral immune responses. From early B cell development in the bone marrow, to their maturation in the periphery, activation in the germinal center, and differentiation into plasma cells or memory B cells, B cells display ever-changing functions and properties. Autophagy and mitochondria play important roles in B cell development, activation, and differentiation to accommodate the phenotypic and environmental changes encountered over the lifetime of the cell...
July 2018: Mitochondrion
Hitendra S Solanki, Niraj Babu, Ankit P Jain, Mohd Younis Bhat, Vinuth N Puttamallesh, Jayshree Advani, Remya Raja, Kiran K Mangalaparthi, Mahesh M Kumar, T S Keshava Prasad, Premendu Prakash Mathur, David Sidransky, Harsha Gowda, Aditi Chatterjee
Cellular transformation owing to cigarette smoking is due to chronic exposure and not acute. However, systematic studies to understand the molecular alterations in lung cells due to cigarette smoke are lacking. To understand these molecular alterations induced by chronic cigarette smoke exposure, we carried out tandem mass tag (TMT) based temporal proteomic profiling of lung cells exposed to cigarette smoke for upto 12months. We identified 2620 proteins in total, of which 671 proteins were differentially expressed (1...
May 2018: Mitochondrion
Jessica L Fetterman, Chunyu Liu, Gary F Mitchell, Ramachandran S Vasan, Emelia J Benjamin, Joseph A Vita, Naomi M Hamburg, Daniel Levy
Mitochondrial genetic variation with resultant alterations in oxidative phosphorylation may influence vascular function and contribute to cardiovascular disease susceptibility. We assessed relations of peptide-encoding variants in the mitochondrial genome with measures of vascular function in Framingham Heart Study participants. Of 258 variants assessed, 40 were predicted to have functional consequences by bioinformatics programs. A maternal pattern of heritability was estimated to contribute to the variability of aortic stiffness...
May 2018: Mitochondrion
Young Joon Kwon, Sujay Guha, Florin Tuluc, Marni J Falk
Mitochondrial respiratory chain disease is caused by a wide range of individually rare genetic disorders that impair cellular energy metabolism. While fluorescence microscopy analysis of nematodes fed MitoTracker Green (MTG) and tetramethylrhodamine ethyl ester (TMRE) can reliably quantify relative mitochondrial density and membrane potential, respectively, in C. elegans models of mitochondrial dysfunction, it is a tedious process with limitations in the number and age of animals that can be studied. A novel, large particle, flow cytometry-based method reported here accelerates and automates the relative quantitation of mitochondrial physiology in nematode populations...
May 2018: Mitochondrion
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