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Amanda C Smith, Yoko Ito, Afsana Ahmed, Jeremy A Schwartzentruber, Chandree L Beaulieu, Erika Aberg, Jacek Majewski, Dennis E Bulman, Karina Horsting-Wethly, Diana Vermunt-de Koning, Richard J Rodenburg, Kym M Boycott, Lynette S Penney
Primary CoQ10 deficiency is a clinically and genetically heterogeneous, autosomal recessive disorder resulting from mutations in genes involved in the synthesis of coenzyme Q10 (CoQ10 ). To date, mutations in nine proteins required for the biosynthesis of CoQ10 cause CoQ10 deficiency with varying clinical presentations. In 2009 the first patient with mutations in COQ9 was reported in an infant with a neonatal-onset, primary CoQ10 deficiency with multi-system disease. Here we describe four siblings with a previously undiagnosed lethal disorder characterized by oligohydramnios and intrauterine growth restriction, variable cardiomyopathy, anemia, and renal anomalies...
March 20, 2018: Journal of Inherited Metabolic Disease
Fan Wang, Xinghua Zhong, Haibin Wang, Aiping Song, Fadi Chen, Weimin Fang, Jiafu Jiang, Nianjun Teng
Most chrysanthemum cultivars are self-incompatible, so it is very difficult to create pure lines that are important in chrysanthemum breeding and theoretical studies. In our previous study, we obtained a self-compatible chrysanthemum cultivar and its self-pollinated seed set was 56.50%. It was interesting that the seed set of its ten progenies ranged from 0% to 37.23%. Examination of the factors causing the differences in the seed set will lead to an improved understanding of chrysanthemum self-incompatibility, and provide valuable information for creating pure lines...
March 13, 2018: International Journal of Molecular Sciences
Roan Louw, Izelle Smuts, Kimmey-Li Wilsenach, Lindi-Maryn Jonck, Maryke Schoonen, Francois H van der Westhuizen
BACKGROUND: Coenzyme Q10 (CoQ10 ) is an important component of the mitochondrial respiratory chain (RC) and is critical for energy production. Although the prevalence of CoQ10 deficiency is still unknown, the general consensus is that the condition is under-diagnosed. The aim of this study was to retrospectively investigate CoQ10 deficiency in frozen muscle specimens in a cohort of ethnically diverse patients who received muscle biopsies for the investigation of a possible RC deficiency (RCD)...
February 23, 2018: Molecular Genetics and Metabolism
Erzsebet Polyak, Julian Ostrovsky, Min Peng, Stephen D Dingley, Mai Tsukikawa, Young Joon Kwon, Shana E McCormack, Michael Bennett, Rui Xiao, Christoph Seiler, Zhe Zhang, Marni J Falk
Oxidative stress is a known contributing factor in mitochondrial respiratory chain (RC) disease pathogenesis. Yet, no efficient means exists to objectively evaluate the comparative therapeutic efficacy or toxicity of different antioxidant compounds empirically used in human RC disease. We postulated that pre-clinical comparative analysis of diverse antioxidant drugs having suggested utility in primary RC disease using animal and cellular models of RC dysfunction may improve understanding of their integrated effects and physiologic mechanisms, and enable prioritization of lead antioxidant molecules to pursue in human clinical trials...
February 23, 2018: Molecular Genetics and Metabolism
Cuicui Mu, Lili Li, Xiaodong Wu, Feng Zhang, Lin Jia, Qian Zhao, Tingjun Zhang
Deep carbon pool in permafrost regions is an important component of the global terrestrial carbon cycle. However, the greenhouse gas production from deep permafrost soils is not well understood. Here, using soils collected from 5-m deep permafrost cores from meadow and wet meadow on the northern Qinghai-Tibetan Plateau (QTP), we investigated the effects of temperature on CO2 and N2 O production under aerobic incubations and CH4 production under anaerobic incubations. After a 35-day incubation, the CO2, N2 O and CH4 production at -2 °C to 10 °C were 0...
March 9, 2018: Scientific Reports
Joao A M Carneiro, Igor F Canisso, R S Bandeira, V F C Scheeren, Camila P Freitas-Dell'Aqua, Marco A Alvarenga, Frederico O Papa, Jose A Dell'Aqua
This study aimed to evaluate the antioxidant properties of coenzyme Q10 (CoQ10) during cryopreservation of semen obtained from stallions having good and bad semen freezing ability (GFA vs. BFA, respectively). Forty ejaculates (n = 20 stallions) were split into five centrifugation and five freezing extenders containing different concentrations of CoQ10 (0, 25, 50, 75 and 100 μmols/L). If CoQ10 was added to the centrifugation extender, the freezing extender had no CoQ10 added; similarly, if CoQ10 was added to the freezing extender, the centrifugation extender had no CoQ10...
February 24, 2018: Animal Reproduction Science
Jamie Perin, Gregory Prokopowicz, Margaret Furtado, Konstantinos Papas, Kimberley E Steele
BACKGROUND: Vitamin and mineral deficiencies are common following Roux-en-Y gastric bypass (RYGB) and can lead to significant morbidity, but little research on the efficacy of vitamin supplementation regimens exists. We compared the efficacy and tolerability of an investigational versus a standard multivitamin regimen in patients undergoing RYGB. METHODS: Fifty-six patients, aged 18 to 65, were randomized to an investigational versus a standard multivitamin. Plasma levels of vitamins A, B-12, D, E-α, E-β/γ, thiamine, folate, iron, iron-binding capacity, iron saturation, prealbumin, and parathyroid hormone (PTH) were measured at 3 and 6 months postoperatively...
March 2, 2018: Obesity Surgery
Ali Emami, Asghar Tofighi, Siamak Asri-Rezaei, Behnaz Bazargani-Gilani
Strenuous physical exercise and hyperthermia may paradoxically induce oxidative stress and adverse effects on myocardial function. The purpose of this study was to investigate the effect of 14-d coenzyme Q10 (CoQ10) supplementation and pre-cooling on serum creatine kinase-MB (CK-MB), cardiac Troponin I (cTnI), myoglobin (Mb), lactate dehydrogenase (LD), total antioxidant capacity (TAC), lipid peroxidation (LPO) and CoQ10 concentration in elite swimmers. In total, thirty-six healthy males (mean age 17 (sd 1) years) were randomly selected and divided into four groups of supplementation, supplementation with pre-cooling, pre-cooling and control...
February 2018: British Journal of Nutrition
Jean-Marc Zingg
The hydrophobicity of vitamin E poses transport and metabolic challenges to regulate its bioavailability and to prevent its accumulation in lipid-rich tissues such as adipose tissue, brain, and liver. Water-soluble precursors of vitamin E (α-tocopherol, αT), such as its esters with acetate (αTA), succinate (αTS), or phosphate (αTP), have increased solubility in water and stability against reaction with free radicals, but they are rapidly converted during their uptake into the lipid-soluble vitamin E. Therefore, the bioavailability of these precursors as intact molecules is low; nevertheless, at least for αTS and αTP, the recent research has revealed unique regulatory effects on signal transduction and gene expression and the modulation of cellular events ranging from proliferation, survival/apoptosis, lipid uptake and metabolism, phagocytosis, long term potentiation, cell migration, telomere maintenance, and angiogenesis...
2018: Advances in Food and Nutrition Research
Wang Qingkui, Shengen Liu, Peng Tian
Understanding the temperature sensitivity (Q10 ) of soil organic C (SOC) decomposition is critical to quantifying the climate-carbon cycle feedback and predicting the response of ecosystems to climate change. However, the driving factors of the spatial variation in Q10 at a continental scale are fully unidentified. In this study, we conducted a novel incubation experiment with periodically varying temperature based on the mean annual temperature of the soil origin sites. A total of 140 soil samples were collected from 22 sites along a 3200 km-long north-south transect of forests in China, and the Q10 of soil microbial respiration and corresponding environmental variables were measured...
February 24, 2018: Global Change Biology
Caitlin E Hicks Pries, C Castanha, R Porras, Claire Phillips, M S Torn
Temperature records and model predictions demonstrate that deep soils warm at the same rate as surface soils, contrary to Xiao et al 's assertions. In response to Xiao et al 's critique of our Q10 analysis, we present the results with all data points included, which show Q10 values of >2 throughout the soil profile, indicating that all soil depths responded to warming.
February 23, 2018: Science
Jing Xiao, Fangjian Yu, Wanying Zhu, Chenchao Xu, Kaihang Zhang, Yiqi Luo, James M Tiedje, Jizhong Zhou, Lei Cheng
In a compelling study, Hicks Pries et al (Reports, 31 March 2017, p. 1420) showed that 4°C warming enhanced soil CO2 production in the 1-meter soil profile, with all soil depths displaying similar temperature sensitivity (Q10 ). We argue that some caveats can be identified in their experimental approach and analysis, and that these critically undermine their conclusions and hence their claim that the strength of feedback between the whole-soil carbon and climate has been underestimated in terrestrial models...
February 23, 2018: Science
Emma K Eriksson, Víctor Agmo Hernández, Katarina Edwards
Ubiquinone-10 (Q10) plays a pivotal role as electron-carrier in the mitochondrial respiratory chain, and is also well known for its powerful antioxidant properties. Recent findings suggest moreover that Q10 could have an important membrane stabilizing function. In line with this, we showed in a previous study that Q10 decreases the permeability to carboxyfluorescein (CF) and increases the mechanical strength of 1-palmitoyl-2-oleyl-sn-glycero-phosphocholine (POPC) membranes. In the current study we report on the effects exerted by Q10 in membranes having a more complex lipid composition designed to mimic that of the inner mitochondrial membrane (IMM)...
February 19, 2018: Biochimica et Biophysica Acta
Thibault Leger, Isabelle Hininger-Favier, Frédéric Capel, Alain Geloen, Jean-Paul Rigaudière, Chrystèle Jouve, Elodie Pitois, Gaelle Pineau, Carole Vaysse, Jean-Michel Chardigny, Marie-Caroline Michalski, Corinne Malpuech-Brugère, Luc Demaison
Background: Obesity progressively leads to cardiac failure. Omega-3 polyunsaturated fatty acids (PUFA) have been shown to have cardio-protective effects in numerous pathological situations. It is not known whether rapeseed oil, which contains α-linolenic acid (ALA), has a similar protective effect. Omega-3 PUFAs are sensitive to attack by reactive oxygen species (ROS), and lipid peroxidation products could damage cardiac cells. We thus tested whether dietary refined rapeseed oil (RSO) associated with or without different antioxidants (vitamin E, coenzyme Q10 and canolol) is cardio-protective in a situation of abdominal obesity...
2018: Nutrition & Metabolism
V Mojto, A Gvozdjakova, J Kucharska, Z Rausova, O Vancova, J Valuch
OBJECTIVE: The aim of the study was to observe the influence of 11-days complete water fasting (WF) and regeneration diet (RD) on renal function, body weight, blood pressure and oxidative stress. BACKGROUND: Therapeutic WF is considered a healing method. METHODS: Ten volunteers drank only water for 11 days, followed by RD for the next 11 days. Data on body weight, blood pressure, kidney functions, antioxidants, lipid peroxidation, cholesterols, triacylglycerols and selected biochemical parameters were obtained...
2018: Bratislavské Lekárske Listy
Peiwen Zhang, Chen Yang, Honghui Guo, Jiaji Wang, Shengqiang Lin, Huamei Li, Yan Yang, Wenhua Ling
BACKGROUND: The use of coenzyme Q10 (CoQ10) as an adjuvant treatment with routine clinical therapy against metabolic diseases has shown benefit. However, the effect of CoQ10 as a primary preventive agent against cardiovascular diseases (CVDs) has not been well studied. OBJECTIVE: The objective of this study was to investigate the effect of CoQ10 on CVD risk factors in dyslipidemic patients. METHODS: In this randomized, double-blinded, placebo-controlled trial, 101 dyslipidemic subjects without taking any hypoglycemic or hypolipidemic drugs were administrated to 120 mg CoQ10 or placebo daily for 24 weeks...
December 21, 2017: Journal of Clinical Lipidology
Carl Erb, Katarzyna Konieczka
Mitochondrial function is closely linked to numerous aspects of eye health. Imbalance between the creation of energy and the development of reactive oxygen species (ROS) seems to be the cause of the development of mitochondrial dysfunctions. As a result of this energy deficit, the level of oxidative stress in the eye tissues increases, leading to numerous ophthalmic impairments. It is important to distinguish between primary mitochondrial eye diseases and secondary mitochondrial changes. Primary mitochondrial eye diseases, for example Leber's hereditary optic atrophy (LHON), retinitis pigmentosa and chronic progressive external ophthalmoplegia are caused by direct damage to mitochondrial function induced by defective genes, either located on mitochondrial DNA (mtDNA) or the DNA of the nucleus (nDNA)...
February 2018: Klinische Monatsblätter Für Augenheilkunde
M Zacchigna, F Cateni, G Procida, D Zampieri, M G Mamolo
Methoxy polyethylene glycol conjugated with coenzyme Q10 (mPEG)-CoQ10 and analog adducts with amino acids as spacers were synthesized as a new drug delivery systems for CoQ10. Alanine and branched chain amino acids (valine, leucine and isoleucine) were conjugated to mPEG by an amide linkage and to CoQ10 by an ester bond. Recently, branched chain amino acids (BCAAs), which are released along with CoQ10, have received increasing attention as 'anti-fatigue' elements. FT-IR and 1H NMR spectroscopic analysis were useful to characterize the synthesized conjugates...
December 1, 2017: Die Pharmazie
Hemanth Noothalapati, Ryo Ikarashi, Keita Iwasaki, Tatsuro Nishida, Tomohiro Kaino, Keisuke Yoshikiyo, Keiji Terao, Daisuke Nakata, Naoko Ikuta, Masahiro Ando, Hiro-O Hamaguchi, Makoto Kawamukai, Tatsuyuki Yamamoto
α-lipoic acid (ALA) is an essential cofactor for many enzyme complexes in aerobic metabolism, especially in mitochondria of eukaryotic cells where respiration takes place. It also has excellent anti-oxidative properties. The acid has two stereo-isomers, R- and S- lipoic acid (R-LA and S-LA), but only the R-LA has biological significance and is exclusively produced in our body. A mutant strain of fission yeast, Δdps1, cannot synthesize coenzyme Q10, which is essential during yeast respiration, leading to oxidative stress...
February 6, 2018: Spectrochimica Acta. Part A, Molecular and Biomolecular Spectroscopy
Iman Yousefian, Mojtaba Emamverdi, Amir Karamzadeh-Dehaghani, Raziyeh Sabzian-Melei, Mahdi Zhandi, Ahmad Zare-Shahneh
The aim of this study was to determine the quality of post-thawed buck spermatozoa by attenuation of cryopreservation-induced oxidative stress using CoQ10, a lipophilic antioxidant. Ejaculates at every sampling period were collected from four Mahabadi bucks, pooled and diluted in soybean lecithin-based extenders containing 0 (negative control, NC), 0.5 (CQ0.05), 1 (CQ1), and 1.5 (CQ1.5) μM CoQ10 and 0.9% (v/v) DMSO (positive control, PC). The diluted semen was gradually cooled to 4 °C, then frozen and stored in liquid nitrogen...
February 9, 2018: Cryobiology
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