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https://www.readbyqxmd.com/read/29781757/molecular-diagnosis-of-coenzyme-q-10-deficiency-an-update
#1
Delia Yubero, Raquel Montero, Carlos Santos-Ocaña, Leonardo Salviati, Placido Navas, Rafael Artuch
Coenzyme Q10 (CoQ) deficiency syndromes comprise a growing number of genetic disorders. While primary CoQ deficiency syndromes are rare diseases, secondary deficiencies have been related to both genetic and environmental conditions, which are the main causes of biochemical CoQ deficiency. The diagnosis is the essential first step for planning future treatment strategies, as the potential treatability of CoQ deficiency is the most critical issue for the patients. Areas covered: While the quickest and most effective tool to define a CoQ-deficient status is its biochemical determination in biological fluids or tissues, this quantification does not provide a definite diagnosis of a CoQ-deficient status nor insight about the genetic etiology of the disease...
May 21, 2018: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/29755918/coq10-a-super-vitamin-review-on-application-and-biosynthesis
#2
REVIEW
Shraddha Shukla, Kashyap Kumar Dubey
Coenzyme Q10 (CoQ) or ubiquinone is found in the biological system which is synthesized by the conjugation of benzoquinone ring with isoprenoid chain of variable length. Coenzyme Q10 supplementation energizes the body and increases body energy production in the form of ATP and helps to treat various human diseases such as cardiomyopathy, muscular dystrophy, periodontal disease, etc. Reports of these potential therapeutic advantages of CoQ10 have resulted in its high market demand, which focus the researchers to work on this molecule and develop better bioprocess methods for commercial level production...
May 2018: 3 Biotech
https://www.readbyqxmd.com/read/29743240/control-of-mitochondrial-superoxide-production-by-reverse-electron-transport-at-complex-i
#3
Ellen L Robb, Andrew R Hall, Tracy A Prime, Simon Eaton, Marten Szibor, Carlo Viscomi, Andrew M James, Michael P Murphy
The generation of mitochondrial superoxide (O2 •- ) by reverse electron transport (RET) at complex I causes oxidative damage in pathologies such as ischemia reperfusion injury, but also provides the precursor to H2 O2 production in physiological mitochondrial redox signaling. Here, we quantified the factors that determine mitochondrial O2 •- production by RET in isolated heart mitochondria. Measuring mitochondrial H2 O2 production at a range of Δp values and for several CoQ and NADH pool redox states obtained with the uncoupler p-trifluoromethoxyphenylhydrazone, we show that O2 •- production by RET responds to changes in O2 concentration, the magnitude of the proton-motive force (Δp), and the redox states of the coenzyme Q (CoQ) and NADH pools...
May 9, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29734881/effect-of-ubiquinol-supplementation-on-biochemical-and-oxidative-stress-indexes-after-intense-exercise-in-young-athletes
#4
Patrick Orlando, Sonia Silvestri, Roberta Galeazzi, Roberto Antonicelli, Fabio Marcheggiani, Ilenia Cirilli, Tiziana Bacchetti, Luca Tiano
OBJECTIVES: Physical exercise significantly impacts the biochemistry of the organism. Ubiquinone is a key component of the mitochondrial respiratory chain and ubiquinol, its reduced and active form, is an emerging molecule in sport nutrition. The aim of this study was to evaluate the effect of ubiquinol supplementation on biochemical and oxidative stress indexes after an intense bout of exercise. METHODS: 21 male young athletes (26 + 5 years of age) were randomized in two groups according to a double blind cross-over study, either supplemented with ubiquinol (200 mg/day) or placebo for 1 month...
December 2018: Redox Report: Communications in Free Radical Research
https://www.readbyqxmd.com/read/29702132/complete-genome-sequence-of-bacillus-velezensis-qst713-a-biocontrol-agent-that-protects-agaricus-bisporus-crops-against-the-green-mould-disease
#5
Caroline Pandin, Dominique Le Coq, Julien Deschamps, Régis Védie, Thierry Rousseau, Stéphane Aymerich, Romain Briandet
Bacillus subtilis QST713 is extensively used as a biological control agent in agricultural fields including in the button mushroom culture, Agaricus bisporus. This last use exploits its inhibitory activity against microbial pathogens such as Trichoderma aggressivum f. europaeum, the main button mushroom green mould competitor. Here, we report the complete genome sequence of this bacterium with a genome size of 4 233 757 bp, 4263 predicted genes and an average GC content of 45.9%. Based on phylogenomic analyses, strain QST713 is finally designated as Bacillus velezensis...
April 24, 2018: Journal of Biotechnology
https://www.readbyqxmd.com/read/29656001/-fl%C3%A3-neur-neurologique-in-paris-a-guide-to-pinpointing-the-houses-of-famous-neurologists-in-the-late-xix-century
#6
REVIEW
Hélio Afonso Ghizoni Teive, Francisco Manoel Branco Germiniani, Carlos Henrique Ferreira Camargo, Olivier Walusinski, Andrew J Lees
INTRODUCTION: During the last quarter of the XIX century, Paris, France, particularly the Salpêtrière Hospital was the most important centre of reference of Clinical Neurology in the world. The group based on the Salpêtrière Hospital, led by Professor Charcot, who was arguably the most celebrated neurologist in Europe. OBJECTIVE: In this historical review, we present and locate the addresses of the houses of these famous Parisian neurologists from the late XIX century...
April 11, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29577973/the-effect-of-n-3-fatty-acids-and-coenzyme-q10-supplementation-on-neutrophil-leukotrienes-mediators-of-inflammation-resolution-and-myeloperoxidase-in-chronic-kidney-disease
#7
Anne E Barden, Sujata Shinde, Valerie Burke, Ian B Puddey, Lawrence J Beilin, Ashley B Irish, Gerald F Watts, Trevor A Mori
BACKGROUND: Neutrophils release leukotriene (LT)B4 and myeloperoxidase (MPO) that may be important mediators of chronic inflammation in chronic kidney disease (CKD). The n-3 fatty acids (n-3 FA) eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) have the potential to attenuate inflammation through production of LTB5 and the Specialized Proresolving Lipid Mediators (SPM) that promote the resolution of inflammation. In animal models, coenzyme Q10 (CoQ) also attenuates inflammation by reducing MPO and LTB4 ...
March 22, 2018: Prostaglandins & Other Lipid Mediators
https://www.readbyqxmd.com/read/29537844/ionic-to-electronic-conductivity-crossover-in-cdte-agi-as-2-te-3-glasses-an-110m-ag-tracer-diffusion-study
#8
M Kassem, I Alekseev, M Bokova, D Le Coq, E Bychkov
Conductivity isotherms of (CdTe) x (AgI)0.5- x/2 (As2 Te3 )0.5- x/2 glasses (0.0 ≤ x ≤ 0.15) reveal a nonmonotonic behavior with increasing CdTe content reminiscent of mixed cation effect in oxide and chalcogenide glasses. Nevertheless, the apparent similarity appears to be partly incorrect. Using 110m Ag tracer diffusion measurements, we show that semiconducting CdTe additions produce a dual effect: (i) decreasing the Ag+ ion transport by a factor of ≈200 with a simultaneous increase of the diffusion activation energy and (ii) increasing the electronic conductivity by 1...
April 12, 2018: Journal of Physical Chemistry. B
https://www.readbyqxmd.com/read/29475487/highly-sensitive-and-selective-determination-of-redox-states-of-coenzymes-q-9-and-q-10-in-mice-tissues-application-of-orbitrap-mass-spectrometry
#9
Renu Pandey, Christopher L Riley, Edward M Mills, Stefano Tiziani
Coenzyme Q (CoQ) is a redox active molecule that plays a fundamental role in mitochondrial energy generation and functions as a potent endogenous antioxidant. Redox ratio of CoQ has been suggested as a good marker of mitochondrial dysfunction and oxidative stress. Nevertheless, simultaneous measurement of redox states of CoQ is challenging owing to its hydrophobicity and instability of the reduced form. In order to improve the analytical methodology, paying special attention to this instability, we developed a highly sensitive and selective high-resolution/accurate-mass (HR/AM) UHPLC-MS/MS method for the rapid determination of redox states of CoQ9 and CoQ10 by ultra-performance liquid chromatography-hybrid quadrupole-Orbitrap mass spectrometry...
June 29, 2018: Analytica Chimica Acta
https://www.readbyqxmd.com/read/29459830/coenzyme-q-10-supplementation-in-aging-and-disease
#10
REVIEW
Juan D Hernández-Camacho, Michel Bernier, Guillermo López-Lluch, Plácido Navas
Coenzyme Q (CoQ) is an essential component of the mitochondrial electron transport chain and an antioxidant in plasma membranes and lipoproteins. It is endogenously produced in all cells by a highly regulated pathway that involves a mitochondrial multiprotein complex. Defects in either the structural and/or regulatory components of CoQ complex or in non-CoQ biosynthetic mitochondrial proteins can result in a decrease in CoQ concentration and/or an increase in oxidative stress. Besides CoQ10 deficiency syndrome and aging, there are chronic diseases in which lower levels of CoQ10 are detected in tissues and organs providing the hypothesis that CoQ10 supplementation could alleviate aging symptoms and/or retard the onset of these diseases...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29452608/insights-into-an-ancient-atypical-kinase-essential-for-biosynthesis-of-coenzyme-q
#11
Catherine F Clarke
COQ8 proteins are homologs of atypical protein kinases required for the biosynthesis of coenzyme Q (CoQ). In this issue of Cell Chemical Biology, Reidenbach et al. (2018) show that COQ8 has an ATPase activity, required for CoQ biosynthesis, that is strongly activated by cardiolipin and small molecule mimics of early CoQ intermediates.
February 15, 2018: Cell Chemical Biology
https://www.readbyqxmd.com/read/29451807/coenzyme-q-10-from-bench-to-clinic-in-aging-diseases-a-translational-review
#12
Francisco M Gutierrez-Mariscal, Elena M Yubero-Serrano, Jose M Villalba, Jose Lopez-Miranda
Coenzyme Q10 (CoQ10 ) is a ubiquitous molecule present in all eukaryotic organisms whose principal role in the cell is related to its participation in the electron transport chain in the inner mitochondrial membrane. CoQ10 plays a major role in the control of cell redox status, and both the amount and functionality of this molecule have been related to the regulation of reactive oxygen species generation. Numerous reports can be found discussing the implications of CoQ10 supplementation in human studies and clinical trials related to aging...
February 16, 2018: Critical Reviews in Food Science and Nutrition
https://www.readbyqxmd.com/read/29444838/enzyme-polymorphism-oxygen-and-injury-a-lipidomic-analysis-of-flight-induced-oxidative-damage-in-a-succinate-dehydrogenase-d-sdhd-polymorphic-insect
#13
Julianne E Pekny, Philip B Smith, James H Marden
When active tissues receive insufficient oxygen to meet metabolic demand, succinate accumulates and has two fundamental effects: it causes ischemia-reperfusion injury while also activating the hypoxia-inducible factor pathway (HIF). The Glanville fritillary butterfly ( Melitaea cinxia ) possesses a balanced polymorphism in Sdhd , shown previously to affect HIF pathway activation and tracheal morphology and used here to experimentally test the hypothesis that variation in succinate dehydrogenase affects oxidative injury ...
March 23, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29443001/short-and-medium-range-structures-of-80gese-2-20ga-2-se-3-chalcogenide-glasses
#14
Elena Petracovschi, Laurent Calvez, Laurent Cormier, David Le Coq, Jincheng Du
The short and medium range structures of 80GeSe2 -20Ga2 Se3 (or Ge23.5 Ga11.8 Se64.7 ) chalcogenide glasses have been studied by combining ab initio molecular dynamics (AIMD) simulations and experimental neutron diffraction studies. The structure factor and total correlation function were calculated from glass structures generated from AIMD simulations and compared with neutron diffraction experiments showing reasonable agreement. The atomic structures of ternary chalcogenide glasses were analyzed in detail, and it was found that gallium atoms are four-fold coordinated by selenium (Se) and form [GaSe4 ] tetrahedra...
May 10, 2018: Journal of Physics. Condensed Matter: An Institute of Physics Journal
https://www.readbyqxmd.com/read/29437246/early-movement-restriction-leads-to-enduring-disorders-in-muscle-and-locomotion
#15
Maxime Delcour, Vicky S Massicotte, Michaël Russier, Hélène Bras, Julie Peyronnet, Marie-Hélène Canu, Florence Cayetanot, Mary F Barbe, Jacques-Olivier Coq
Motor control and body representation in the central nervous system (CNS) as well as musculoskeletal architecture and physiology are shaped during development by sensorimotor experience and feedback, but the emergence of locomotor disorders during maturation and their persistence over time remain a matter of debate in the absence of brain damage. By using transient immobilization of the hind limbs, we investigated the enduring impact of postnatal sensorimotor restriction (SMR) on gait and posture on treadmill, age-related changes in locomotion, musculoskeletal histopathology and Hoffmann reflex in adult rats without brain damage...
February 13, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29430932/experimental-and-theoretical-insights-into-the-structure-of-tellurium-chloride-glasses
#16
Lila Bouëssel Du Bourg, Eric Furet, Alicia Lecomte, Laurent Le Pollès, Shinji Kohara, Chris J Benmore, Eugène Bychkov, David Le Coq
The structure of the binary chalcohalide glasses Te1- x Cl x (0.35 ≤ x ≤ 0.65) is considered by combining experimental and theoretical results. The structural network properties are influenced by a competition between ionic and covalent bonding in such glasses. At first, a focus is placed on the detailed information available by using the complementary high-energy X-ray and the neutron diffractions in both the reciprocal and real spaces. The main characteristic suggested by the structure factors S( Q) concerns the presence of three length scales in the intermediate range order...
March 5, 2018: Inorganic Chemistry
https://www.readbyqxmd.com/read/29402381/mitochondrial-coq-deficiency-is-a-common-driver-of-mitochondrial-oxidants-and-insulin-resistance
#17
Daniel J Fazakerley, Rima Chaudhuri, Pengyi Yang, Ghassan J Maghzal, Kristen C Thomas, James R Krycer, Sean J Humphrey, Benjamin L Parker, Kelsey H Fisher-Wellman, Christopher C Meoli, Nolan J Hoffman, Ciana Diskin, James G Burchfield, Mark J Cowley, Warren Kaplan, Zora Modrusan, Ganesh Kolumam, Jean Yh Yang, Daniel L Chen, Dorit Samocha-Bonet, Jerry R Greenfield, Kyle L Hoehn, Roland Stocker, David E James
Insulin resistance in muscle, adipocytes and liver is a gateway to a number of metabolic diseases. Here, we show a selective deficiency in mitochondrial coenzyme Q (CoQ) in insulin-resistant adipose and muscle tissue. This defect was observed in a range of in vitro insulin resistance models and adipose tissue from insulin-resistant humans and was concomitant with lower expression of mevalonate/CoQ biosynthesis pathway proteins in most models. Pharmacologic or genetic manipulations that decreased mitochondrial CoQ triggered mitochondrial oxidants and insulin resistance while CoQ supplementation in either insulin-resistant cell models or mice restored normal insulin sensitivity...
February 6, 2018: ELife
https://www.readbyqxmd.com/read/29387172/engineering-yarrowia-lipolytica-to-enhance-lipid-production-from-lignocellulosic-materials
#18
Xochitl Niehus, Anne-Marie Crutz-Le Coq, Georgina Sandoval, Jean-Marc Nicaud, Rodrigo Ledesma-Amaro
Background: Yarrowia lipolytica is a common biotechnological chassis for the production of lipids, which are the preferred feedstock for the production of fuels and chemicals. To reduce the cost of microbial lipid production, inexpensive carbon sources must be used, such as lignocellulosic hydrolysates. Unfortunately, lignocellulosic materials often contain toxic compounds and a large amount of xylose, which cannot be used by Y. lipolytica . Results: In this work, we engineered this yeast to efficiently use xylose as a carbon source for the production of lipids by overexpressing native genes...
2018: Biotechnology for Biofuels
https://www.readbyqxmd.com/read/29364276/a-rat-model-of-mild-intrauterine-hypoperfusion-with-microcoil-stenosis
#19
Masahiro Tsuji, Jacques-Olivier Coq, Yuko Ogawa, Yumi Yamamoto, Makiko Ohshima
Intrauterine hypoperfusion/ischemia is one of the major causes of intrauterine/fetal growth restriction, preterm birth, and low birth weight. Most studies of this phenomenon have been performed in either models with severe intrauterine ischemia or models with gradient degree of intrauterine hypoperfusion. No study has been performed in a model on uniform mild intrauterine hypoperfusion (MIUH). Two models have been used for studies of MIUH: a model based on suture ligation of either side of the arterial arcade formed with the uterine and ovarian arteries, and a transient model based on clipping the bilateral ovarian arteries and aorta having patency...
January 7, 2018: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29361719/antioxidant-and-cytoprotective-effects-of-the-di-o-caffeoylquinic-acid-family-the-mechanism-structure-activity-relationship-and-conformational-effect
#20
Xican Li, Ke Li, Hong Xie, Yulu Xie, Yueying Li, Xiaojun Zhao, Xiaohua Jiang, Dongfeng Chen
In this study, a series of di- O -caffeoylquinic acids ( di-COQs ) were systematically investigated for their antioxidant and cytoprotective effects towards •OH-damaged bone marrow-derived mesenchymal stem cells (bmMSCs). Five di-COQs were measured using a set of antioxidant assays. The results show that adjacent 4,5-Di- O -caffeoylquinic acid ( 4 , 5-COQ ) and 3,4-di- O -caffeoylquinic acid ( 3 , 4-COQ ) always gave lower IC50 values than did non-adjacent di-COQs . In the Fe2+ -chelating assay, 4 , 5-COQ and 3 , 4-COQ presented greater UV-Vis spectra and darker colors than did non-adjacent di-COQs ...
January 20, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
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