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CoQ10 AND Mitochondria

Tobias Hermle, Daniela A Braun, Martin Helmstädter, Tobias B Huber, Friedhelm Hildebrandt
Steroid-resistant nephrotic syndrome is characterized by podocyte dysfunction. Drosophila garland cell nephrocytes are podocyte-like cells and thus provide a potential in vivo model in which to study the pathogenesis of nephrotic syndrome. However, relevant pathomechanisms of nephrotic syndrome have not been studied in nephrocytes. Here, we discovered that two Drosophila slit diaphragm proteins, orthologs of the human genes encoding nephrin and nephrin-like protein 1, colocalize within a fingerprint-like staining pattern that correlates with ultrastructural morphology...
December 8, 2016: Journal of the American Society of Nephrology: JASN
Christian Bergamini, Noah Moruzzi, Francesco Volta, Laura Faccioli, Jantje Gerdes, Maria Cristina Mondardini, Romana Fato
Propofol (2,6-diisopropylphenol) is an anaesthetic widely used for human sedation. Due to its intrinsic antioxidant properties, rapid induction of anaesthesia and fast recovery, it is employed in paediatric anaesthesia and in the intensive care of premature infants. Recent studies have pointed out that exposure to anaesthesia in the early stage of life might be responsible of long-lasting cognitive impairment. The apoptotic neurodegeneration induced by general anaesthetics (GA) involves mitochondrial impairment due to the inhibition of the OXPHOS machinery...
August 2016: Journal of Bioenergetics and Biomembranes
Fuminori Hyodo, Shinji Ito, Hinako Eto, Tomoko Nakaji, Keiji Yasukawa, Ryoma Kobayashi, Hideo Utsumi
Redox metabolism plays a central role in maintaining homeostasis in living organisms. The electron transfer system in mitochondria produces ATP via endogenous redox molecules such as flavin mononucleotide (FMN), flavin adenine dinucleotide (FAD), and coenzyme Q10 (CoQ10), which have flavin or quinone moieties. One-electron transfer reactions convert FMN, FAD, and CoQ10 to the free radical intermediates FMNH and FADH, and CoQ10H, respectively. Dynamic nuclear polarization-magnetic resonance imaging (DNP-MRI) allows us to visualize free radicals in vitro and in vivo...
2016: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
Jocelyn Kim, Anagha Medsinge, Bharesh Chauhan, Cara Wiest, Hannah Scanga, Rachael Monaghan, William H Moore, Ken K Nischal
PURPOSE: Corneal involvement in mitochondrial disease is seldom described. Kearns-Sayre syndrome (KSS) is a mitochondrial disorder characterized by retinitis pigmentosa, external ophthalmoplegia, and heart block. We report 2 patients with KSS with corneal lesions involving the endothelium, which improved with Coenzyme Q10 (CoQ10). Based on recent research regarding the role of dysfunctional oxidative metabolism in Fuchs Endothelial Corneal Dystrophy (FECD), we propose that mitochondrial diseases and FECD share a final pathway...
September 2016: Cornea
Hsiu-Chuan Yen, Yi-Chun Liu, Chia-Chi Kan, Hsing-Ju Wei, Szu-Hsien Lee, Yau-Huei Wei, Yu-Hsiu Feng, Chih-Wei Chen, Chin-Chang Huang
BACKGROUND: The Coq protein complex assembled from several Coq proteins is critical for coenzyme Q6 (CoQ6) biosynthesis in yeast. Secondary CoQ10 deficiency is associated with mitochondrial DNA (mtDNA) mutations in patients. We previously demonstrated that carbonyl cyanide-p-trifluoromethoxyphenylhydrazone (FCCP) suppressed CoQ10 levels and COQ5 protein maturation in human 143B cells. METHODS: This study explored the putative COQ protein complex in human cells through two-dimensional blue native-polyacrylamide gel electrophoresis and Western blotting to investigate its status in 143B cells after FCCP treatment and in cybrids harboring the mtDNA mutation that caused myoclonic epilepsy with ragged-red fibers (MERRF) syndrome...
September 2016: Biochimica et Biophysica Acta
Manuel Jesús Acosta, Luis Vazquez Fonseca, Maria Andrea Desbats, Cristina Cerqua, Roberta Zordan, Eva Trevisson, Leonardo Salviati
Coenzyme Q (CoQ, or ubiquinone) is a remarkable lipid that plays an essential role in mitochondria as an electron shuttle between complexes I and II of the respiratory chain, and complex III. It is also a cofactor of other dehydrogenases, a modulator of the permeability transition pore and an essential antioxidant. CoQ is synthesized in mitochondria by a set of at least 12 proteins that form a multiprotein complex. The exact composition of this complex is still unclear. Most of the genes involved in CoQ biosynthesis (COQ genes) have been studied in yeast and have mammalian orthologues...
August 2016: Biochimica et Biophysica Acta
Bethany J Jenkins, Thomas M Daly, Joanne M Morrisey, Michael W Mather, Akhil B Vaidya, Lawrence W Bergman
Coenzyme Q (CoQ, ubiquinone) is a central electron carrier in mitochondrial respiration. CoQ is synthesized through multiple steps involving a number of different enzymes. The prevailing view that the CoQ used in respiration exists as a free pool that diffuses throughout the mitochondrial inner membrane bilayer has recently been challenged. In the yeast Saccharomyces cerevisiae, deletion of the gene encoding Coq10p results in respiration deficiency without inhibiting the synthesis of CoQ, suggesting that the Coq10 protein is critical for the delivery of CoQ to the site(s) of respiration...
2016: PloS One
Abhinav Sharma, Gregg C Fonarow, Javed Butler, Justin A Ezekowitz, G Michael Felker
Heart failure (HF) with either preserved or reduced ejection fraction is associated with increased morbidity and mortality. Evidence-based therapies are often limited by tolerability, hypotension, electrolyte disturbances, and renal dysfunction. Coenzyme Q10 (CoQ10) may represent a safe therapeutic option for patients with HF. CoQ10 is a highly lipophilic molecule with a chemical structure similar to vitamin K. Although being a common component of cellular membranes, CoQ10's most prominent role is to facilitate the production of adenosine triphosphate in the mitochondria by participating in redox reactions within the electron transport chain...
April 2016: Circulation. Heart Failure
Daniel Schniertshauer, Sonja Müller, Tobias Mayr, Tanja Sonntag, Daniel Gebhard, Jörg Bergemann
Human skin is exposed to a number of harmful agents of which the ultraviolet (UV) component of solar radiation is most important. UV-induced damages include direct DNA lesions as well as oxidative damage in DNA, proteins and lipids caused by reactive oxygen species (ROS). Being the main site of ROS generation in the cell, mitochondria are particularly affected by photostress. The resulting mitochondrial dysfunction may have negative effects on many essential cellular processes. To counteract these effects, coenzyme Q10 (CoQ10 ) is used as a potent therapeutic in a number of diseases...
May 2016: Photochemistry and Photobiology
Francesco Emma, Giovanni Montini, Samir M Parikh, Leonardo Salviati
Mitochondria are increasingly recognized as key players in genetic and acquired renal diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by tubular defects, but glomerular, tubulointerstitial and cystic diseases have also been described. For example, defects in coenzyme Q10 (CoQ10) biosynthesis and the mitochondrial DNA 3243 A>G mutation are important causes of focal segmental glomerulosclerosis in children and in adults, respectively. Although they sometimes present with isolated renal findings, mitochondrial diseases are frequently associated with symptoms related to central nervous system and neuromuscular involvement...
May 2016: Nature Reviews. Nephrology
Garth L Nicolson
Loss of function in mitochondria, the key organelle responsible for cellular energy production, can result in the excess fatigue and other symptoms that are common complaints in almost every chronic disease. At the molecular level, a reduction in mitochondrial function occurs as a result of the following changes: (1) a loss of maintenance of the electrical and chemical transmembrane potential of the inner mitochondrial membrane, (2) alterations in the function of the electron transport chain, or (3) a reduction in the transport of critical metabolites into mitochondria...
August 2014: Integrative Medicine
Sibu P Saha, Thomas F Whayne
Coenzyme Q-10 (CoQ10) is a widely used alternative medication or dietary supplement and one of its roles is as an antioxidant. It naturally functions as a coenzyme and component of oxidative phosphorylation in mitochondria. Decreased levels have been demonstrated in diseased myocardium and in Parkinson disease. Farnesyl pyrophosphate is a critical intermediate for CoQ10 synthesis and blockage of this step may be important in statin myopathy. Deficiency of CoQ10 also has been associated with encephalomyopathy, severe infantile multisystemic disease, cerebellar ataxia, nephrotic syndrome, and isolated myopathy...
January 2016: Southern Medical Journal
Hsiao-Ya Tsai, Chih-Pei Lin, Po-Hsun Huang, Szu-Yuan Li, Jia-Shiong Chen, Feng-Yen Lin, Jaw-Wen Chen, Shing-Jong Lin
Coenzyme Q10 (CoQ10), an antiapoptosis enzyme, is stored in the mitochondria of cells. We investigated whether CoQ10 can attenuate high glucose-induced endothelial progenitor cell (EPC) apoptosis and clarified its mechanism. EPCs were incubated with normal glucose (5 mM) or high glucose (25 mM) environment for 3 days, followed by treatment with CoQ10 (10 μM) for 24 hr. Cell proliferation, nitric oxide (NO) production, and JC-1 assay were examined. The specific signal pathways of AMP-activated protein kinase (AMPK), eNOS/Akt, and heme oxygenase-1 (HO-1) were also assessed...
2016: Journal of Diabetes Research
Yoshihito Kishita, Aleksandra Pajak, Nikhita Ajit Bolar, Carlo M T Marobbio, Camilla Maffezzini, Daniela V Miniero, Magnus Monné, Masakazu Kohda, Henrik Stranneheim, Kei Murayama, Karin Naess, Nicole Lesko, Helene Bruhn, Arnaud Mourier, Rolf Wibom, Inger Nennesmo, Ann Jespers, Paul Govaert, Akira Ohtake, Lut Van Laer, Bart L Loeys, Christoph Freyer, Ferdinando Palmieri, Anna Wredenberg, Yasushi Okazaki, Anna Wedell
S-adenosylmethionine (SAM) is the predominant methyl group donor and has a large spectrum of target substrates. As such, it is essential for nearly all biological methylation reactions. SAM is synthesized by methionine adenosyltransferase from methionine and ATP in the cytoplasm and subsequently distributed throughout the different cellular compartments, including mitochondria, where methylation is mostly required for nucleic-acid modifications and respiratory-chain function. We report a syndrome in three families affected by reduced intra-mitochondrial methylation caused by recessive mutations in the gene encoding the only known mitochondrial SAM transporter, SLC25A26...
November 5, 2015: American Journal of Human Genetics
Yan-Kun Yang, Lin-Ping Wang, Lei Chen, Xiu-Ping Yao, Kun-Qi Yang, Ling-Gen Gao, Xian-Liang Zhou
Advancing age is a major risk factor for the development of cardiovascular diseases. The aetiology of several cardiovascular disorders is thought to involve impaired mitochondrial function and oxidative stress. Coenzyme Q10 (CoQ10) acts as both an antioxidant and as an electron acceptor at the level of the mitochondria. Furthermore, in cardiac patients, plasma CoQ10 has been found to be an independent predictor of mortality. Based on the fundamental role of CoQ10 in mitochondrial bioenergetics and its well-acknowledged antioxidant properties, several clinical trials evaluating CoQ10 have been undertaken in cardiovascular disorders of ageing including chronic heart failure, hypertension, and endothelial dysfunction...
October 23, 2015: Clinica Chimica Acta; International Journal of Clinical Chemistry
Yuma Yamada, Kohei Nakamura, Jiro Abe, Mamoru Hyodo, Sanae Haga, Michitaka Ozaki, Hideyoshi Harashima
We herein report on a mitochondrial therapeutic effect based on the delivery of coenzyme Q10 (CoQ10), an anti-oxidant, to in vivo mitochondria using a MITO-Porter, a liposome-based mitochondrial delivery system that functions via membrane fusion. To evaluate the effects, we used a mouse liver ischemia/reperfusion injury (I/R injury) model, in which mitochondrial reactive oxygen species are overexpressed. We packaged CoQ10 in the lipid phase of a MITO-Porter and optimized the mitochondrial fusogenic activities to produce the CoQ10-MITO-Porter...
September 10, 2015: Journal of Controlled Release: Official Journal of the Controlled Release Society
Assaf Ben-Meir, Shlomi Yahalomi, Brit Moshe, Yoel Shufaro, Benjamin Reubinoff, Ann Saada
OBJECTIVE: To examine coenzyme Q10 (CoQ10)-dependent mitochondrial respiratory chain (MRC) activity in granulosa cells (GC) with aging and examine the effect of in vitro CoQ supplementation. DESIGN: Experimental study. SETTING: Hospital laboratory. PATIENT(S): Ten younger (<32 years) and 10 older (>39 years) patients undergoing in vitro fertilization (IVF) treatment. INTERVENTION(S): Measurement of succinate-cytochrome c reductase (MRC complex II + III) activity in the presence and absence of CoQ1 (a soluble CoQ analog)...
September 2015: Fertility and Sterility
Claudio Asencio, María A Rodríguez-Hernandez, Paz Briones, Julio Montoya, Ana Cortés, Sonia Emperador, Angela Gavilán, Eduardo Ruiz-Pesini, Dèlia Yubero, Raquel Montero, Mercedes Pineda, María M O'Callaghan, María Alcázar-Fabra, Leonardo Salviati, Rafael Artuch, Plácido Navas
Coenzyme Q10 (CoQ10) deficiency is associated to a variety of clinical phenotypes including neuromuscular and nephrotic disorders. We report two unrelated boys presenting encephalopathy, ataxia, and lactic acidosis, who died with necrotic lesions in different areas of brain. Levels of CoQ10 and complex II+III activity were increased in both skeletal muscle and fibroblasts, but it was a consequence of higher mitochondria mass measured as citrate synthase. In fibroblasts, oxygen consumption was also increased, whereas steady state ATP levels were decreased...
March 2016: European Journal of Human Genetics: EJHG
Afshin Mohammadi-Bardbori, Asma Najibi, Najmeh Amirzadegan, Raziyeh Gharibi, Ayat Dashti, Mahmoud Omidi, Arastoo Saeedi, Ali Ghafarian-Bahreman, Hossein Niknahad
CoQ10 shares a biosynthetic pathway with cholesterol therefore it can be a potential target of the widely available lipid-lowering agents such as statins. Statins are the most widely prescribed cholesterol-lowering drugs with the ability to inhibit HMG-CoA (3-hydroxy-3-methylglutaryl-coenzyme A) reductase. Preclinical and clinical safety data have shown that statins do not cause serious adverse effects in humans. However, their long-term administration is associated with a variety of myopatic complaints. The aim of this study was to investigate whether CoQ10 supplementation of animals under high fat diet (HFD) treated with statins is able to bypass the mitochondrial metabolic defects or not? Animals were divided into 7 groups and fed with either regular (RD) or HFD during experiments...
September 5, 2015: European Journal of Pharmacology
Maria Apostolopoulou, Alberto Corsini, Michael Roden
BACKGROUND: Statins inhibit hydroxymethylglutaryl-coenzyme A reductase, decrease plasma low-density lipoprotein cholesterol and reduce cardiovascular morbidity and mortality. They can also exert adverse effects, mostly affecting skeletal muscle, ranging from mild myalgia to rhabdomyolysis. MATERIALS AND METHODS: Based on a PubMed search until December 2014, this review summarizes studies on statin effects on muscle mitochondrial morphology and function in the context of myopathy...
July 2015: European Journal of Clinical Investigation
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