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Coenzyme q

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https://www.readbyqxmd.com/read/29684771/coenzyme-q-10-supplementation-reduces-oxidative-stress-and-decreases-antioxidant-enzyme-activity-in-children-with-autism-spectrum-disorders
#1
Elham Mousavinejad, Mohammad Ali Ghaffari, Forough Riahi, Maryam Hajmohammadi, Zeinab Tiznobeyk, Masoumeh Mousavinejad
Antioxidants and oxidative stress can participate in pathobiochemical mechanisms of autism spectrum disorders (ASDs). The aim was to identify the effects of early CoQ10 supplementation on oxidative stress in children with ASDs. Ninety children with ASDs were included in this study, based on DSM-IV criteria and using Childhood Autism Rating Scale (CARS) scores. Concentrations of CoQ10 , MDA, total antioxidant status (TAS) assay, and antioxidant enzymes (superoxide dismutase or SOD and glutathione peroxidase or GPx) activity were determined in serum before and after 100 days of supportive therapy with CoQ10 at daily doses of 30 and 60 mg...
April 4, 2018: Psychiatry Research
https://www.readbyqxmd.com/read/29623830/the-effects-of-coenzyme-q10-supplementation-on-lipid-profiles-among-patients-with-metabolic-diseases-a-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#2
Nasrin Sharifi, Reza Tabrizi, Mahmood Moosazadeh, Naghmeh Mirhosseini, Kamran B Lankarani, Maryam Akbari, Maryam Chamani, Fariba Kolahdooz, Zatollah Asemi
BACKGROUND AND OBJECTIVE: Oxidative stress and inflammation are key parameters in developing metabolic disorders. Hence, antioxidant intake might be an appropriate approach. Several studies have evaluated the effect of coenzyme Q10 (CoQ10) supplementation on lipid profile among patients with metabolic diseases, though findings are controversial. The aim of this systematic review and meta-analysis was to determine the effects of CoQ10 supplementation on lipid profile in patients with metabolic disorders...
April 5, 2018: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/29577824/cavitating-leukoencephalopathy-with-posterior-predominance-caused-by-a-deletion-in-the-apopt1-gene-in-an-indian-boy
#3
Suvasini Sharma, Preeti Singh, Erika Fernandez-Vizarra, Massimo Zeviani, Marjo S Van der Knaap, Ravindra Kumar Saran
A 5-year-old Indian boy presented with subacute onset regression of milestones associated with seizures and spasticity. The symptoms started after an attack of measles. The magnetic resonance imaging (MRI) of the brain showed cavitating leukodystrophy with posterior predominance. Molecular analysis of the APOPT1 gene, a recently described gene associated with mitochondrial leukodystrophy, showed the patient to be homozygous for a 12.82-kilobase deletion, including coding exon 3. Deletion of exon 3 produces a frameshift, predicting the translation of a truncated protein (p...
January 1, 2018: Journal of Child Neurology
https://www.readbyqxmd.com/read/29574631/oxidative-stress-as-a-possible-mechanism-of-statin-induced-myopathy
#4
REVIEW
Yasin Ahmadi, Amir Ghorbanihaghjo, Mohsen Naghi-Zadeh, Neda Lotfi Yagin
Statins, inhibitors of hydroxy methyl glutaryl coenzyme-A (HMG-CoA) reductase, are the most widely used drugs for treating hypercholesterolemia. However, statins can cause disabling myopathy as their main adverse effect. Several molecular mechanisms underlie the statin-induced myopathy including the decrease in the levels of essential mevalonate and cholesterol derivatives. This review discusses a further mechanism involving the loss of other anti-oxidant defenses besides ubiquinone (Co-Q) in skeletal muscles which produce a significant amount of reactive oxygen species (ROS)...
March 24, 2018: Inflammopharmacology
https://www.readbyqxmd.com/read/29560582/a-family-segregating-lethal-neonatal-coenzyme-q-10-deficiency-caused-by-mutations-in-coq9
#5
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
https://www.readbyqxmd.com/read/29530532/the-dilemma-of-diagnosing-coenzyme-q-10-deficiency-in-muscle
#6
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
https://www.readbyqxmd.com/read/29526616/n-acetylcysteine-and-vitamin-e-rescue-animal-longevity-and-cellular-oxidative-stress-in-pre-clinical-models-of-mitochondrial-complex-i-disease
#7
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
https://www.readbyqxmd.com/read/29510674/evolutionary-analysis-of-mitochondrially-encoded-proteins-of-toad-headed-lizards-phrynocephalus-along-an-altitudinal-gradient
#8
Yuanting Jin, Yubin Wo, Haojie Tong, Sen Song, Lixun Zhang, Richard P Brown
BACKGROUND: Animals living at high altitude must adapt to environments with hypoxia and low temperatures, but relatively little is known about underlying genetic changes. Toad-headed lizards of the genus Phrynocephalus cover a broad altitudinal gradient of over 4000 m and are useful models for studies of such adaptive responses. In one of the first studies to have considered selection on mitochondrial protein-coding regions in an ectothermic group distributed over such a wide range of environments, we analysed nineteen complete mitochondrial genomes from all Chinese Phrynocephalus (including eight genomes sequenced for the first time)...
March 6, 2018: BMC Genomics
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/29469215/dinitrosyl-iron-complexes-formation-and-antiradical-action-in-heart-mitochondria
#10
Konstantin B Shumaev, Arina L Dudylina, Marina V Ivanova, Igor S Pugachenko, Enno K Ruuge
Mitochondria are widely known as a major source of reactive oxygen and nitrogen species for the cardiovascular system. Numerous studies established that superoxide anion radical production by heart mitochondria is only slightly suppressed under conditions of deep hypoxia, but is completely blocked under anoxia. It was found also that dinitrosyl iron complexes (DNIC) compare favourably with other physiologically active derivatives of nitric oxide (NO). DNIC with glutathione effectively scavenge superoxide radicals generated by mitochondria at different partial pressures of oxygen...
February 22, 2018: BioFactors
https://www.readbyqxmd.com/read/29459830/coenzyme-q-10-supplementation-in-aging-and-disease
#11
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
#12
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/29452236/cardiac-and-placental-mitochondrial-characterization-in-a-rabbit-model-of-intrauterine-growth-restriction
#13
M Guitart-Mampel, A Gonzalez-Tendero, S Niñerola, C Morén, M Catalán-Garcia, I González-Casacuberta, D L Juárez-Flores, O Ugarteburu, L Matalonga, M V Cascajo, F Tort, A Cortés, E Tobias, J C Milisenda, J M Grau, F Crispi, E Gratacós, G Garrabou, F Cardellach
BACKGROUND: Intrauterine growth restriction (IUGR) is associated with cardiovascular remodeling persisting into adulthood. Mitochondrial bioenergetics, essential for embryonic development and cardiovascular function, are regulated by nuclear effectors as sirtuins. A rabbit model of IUGR and cardiovascular remodeling was generated, in which heart mitochondrial alterations were observed by microscopic and transcriptomic analysis. We aimed to evaluate if such alterations are translated at a functional mitochondrial level to establish the etiopathology and potential therapeutic targets for this obstetric complication...
February 13, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29451807/coenzyme-q-10-from-bench-to-clinic-in-aging-diseases-a-translational-review
#14
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-sdh-polymorphic-insect
#15
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 ...
February 14, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29428005/effects-of-branched-chain-volatile-fatty-acids-on-lactation-performance-and-mrna-expression-of-genes-related-to-fatty-acid-synthesis-in-mammary-gland-of-dairy-cows
#16
Q Liu, C Wang, G Guo, W J Huo, S L Zhang, C X Pei, Y L Zhang, H Wang
Branched-chain volatile fatty acids (BCVFA) supplements could promote lactation performance and milk quality by improving ruminal fermentation and milk fatty acid synthesis. This study was conducted to evaluate the effects of BCVFA supplementation on milk performance, ruminal fermentation, nutrient digestibility and mRNA expression of genes related to fatty acid synthesis in mammary gland of dairy cows. A total of 36 multiparous Chinese Holstein cows averaging 606±4.7 kg of BW, 65±5.2 day in milk (DIM) with daily milk production of 30...
February 12, 2018: Animal: An International Journal of Animal Bioscience
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/29361595/fasting-enhances-mitochondrial-efficiency-in-duckling-skeletal-muscle-by-acting-on-the-substrate-oxidation-system
#18
Damien Roussel, Mélanie Boël, Caroline Romestaing
During food deprivation, animals must develop physiological responses to maximize energy conservation and survival. At the subcellular level, energy conservation is mainly achieved by a reduction in mitochondrial activity and an upregulation of oxidative phosphorylation efficiency. The aim of this study was to decipher mechanisms underlying the increased mitochondrial coupling efficiency reported in fasted birds. Mitochondrial oxidative phosphorylation activity, efficiency and membrane potential were measured in mitochondria isolated from the gastrocnemius muscle of ducklings...
February 14, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29330704/the-effects-of-coenzyme-q10-supplementation-on-blood-pressures-among-patients-with-metabolic-diseases-a-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#19
Reza Tabrizi, Maryam Akbari, Nasrin Sharifi, Kamran B Lankarani, Mahmood Moosazadeh, Fariba Kolahdooz, Mohsen Taghizadeh, Zatollah Asemi
INTRODUCTION: Although several trials have assessed the effect of coenzyme Q10 (CoQ10) supplementation on blood pressures among patients with metabolic diseases, findings are controversial. AIM: This review of randomized controlled trials (RCTs) was performed to summarize the evidence on the effects of CoQ10 supplementation on blood pressures among patients with metabolic diseases. METHODS: Randomized-controlled trials (RCTs) published in PubMed, EMBASE, Web of Science and Cochrane Library databases up to 10 August 2017 were searched...
March 2018: High Blood Pressure & Cardiovascular Prevention: the Official Journal of the Italian Society of Hypertension
https://www.readbyqxmd.com/read/29319808/intracellular-reduction-of-coenzyme-q-homologues-with-a-short-isoprenoid-side-chain-induces-apoptosis-of-hela-cells
#20
Takayuki Takahashi, Yukitoshi Mine, Tadashi Okamoto
Coenzyme Q (CoQ) is an essential factor of the mitochondrial respiratory chain. CoQ homologues with different lengths of the isoprenoid side chain are widely distributed in nature, but little is known about the relationship between the isoprenoid side chain length and biological function; therefore, we examined the effects of CoQ homologues on HeLa cells. When CoQ homologues with a shorter isoprenoid side chain than CoQ4 were added to HeLa cells, they induced cell death, and the order of cytotoxic intensity was as follows: CoQ0 ≫ CoQ3 ≈ CoQ1 > CoQ2 ≫ CoQ4...
April 1, 2018: Journal of Biochemistry
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