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https://www.readbyqxmd.com/read/28792888/structural-basis-for-interdomain-communication-in-ship2-providing-high-phosphatase-activity
#1
Johanne Le Coq, Marta Camacho-Artacho, José Vicente Velázquez, Clara M Santiveri, Luis Heredia Gallego, Ramón Campos-Olivas, Nicole Dölker, Daniel Lietha
SH2-containing-inositol-5-phosphatases (SHIPs) dephosphorylate the 5-phosphate of phosphatidylinositol-3,4,5-trisphosphate (PI(3,4,5)P3) and play important roles in regulating the PI3K/Akt pathway in physiology and disease. Aiming to uncover interdomain regulatory mechanisms in SHIP2, we determined crystal structures containing the 5-phosphatase and a proximal region adopting a C2 fold. This reveals an extensive interface between the two domains, which results in significant structural changes in the phosphatase domain...
August 9, 2017: ELife
https://www.readbyqxmd.com/read/28790927/the-role-of-sulfide-oxidation-impairment-in-the-pathogenesis-of-primary-coq-deficiency
#2
REVIEW
Catarina M Quinzii, Marta Luna-Sanchez, Marcello Ziosi, Agustin Hidalgo-Gutierrez, Giulio Kleiner, Luis C Lopez
Coenzyme Q (CoQ) is a lipid present in all cell membranes. One of the multiple metabolic functions of CoQ is to transport electrons in the reaction catalyzed by sulfide:quinone oxidoreductase (SQOR), the first enzyme of the oxidation pathway of sulfides (hydrogen sulfide, H2S). Early evidence of a defect in the metabolism of H2S in primary CoQ deficiency came from yeast studies in Schizosaccharomyces pombe strains defective for dps1 and ppt1 (homologs of PDSS1 and COQ2, respectively), which have H2S accumulation...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28736527/human-coq9-rescues-a-coq9-yeast-mutant-by-enhancing-coenzyme-q-biosynthesis-from-4-hydroxybenzoic-acid-and-stabilizing-the-coq-synthome
#3
Cuiwen H He, Dylan S Black, Christopher M Allan, Brigitte Meunier, Shamima Rahman, Catherine F Clarke
Coq9 is required for the stability of a mitochondrial multi-subunit complex, termed the CoQ-synthome, and the deamination step of Q intermediates that derive from para-aminobenzoic acid (pABA) in yeast. In human, mutations in the COQ9 gene cause neonatal-onset primary Q10 deficiency. In this study, we determined whether expression of human COQ9 could complement yeast coq9 point or null mutants. We found that expression of human COQ9 rescues the growth of the temperature-sensitive yeast mutant, coq9-ts19, on a non-fermentable carbon source and increases the content of Q6, by enhancing Q biosynthesis from 4-hydroxybenzoic acid (4HB)...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28736036/-regulation-of-the-expression-of-coenzyme-q-synthesis-complex-during-ageing
#4
Carmen Campos-Silva, Iván Reyes-Torres, Maximiliano Rivera, Catherine Meza-Torres, Juan Diego Hernández-Camacho, Elisabet Rodríguez-Bies, Plácido Navas, Guillermo López-Lluch
INTRODUCTION: Coenzyme Q is an essential component in the activity of the mitochondrial electron transport chain. Its synthesis involves, at least, a complex of ten different proteins. In this study, an attempt is made to determine the evolution of the expression of the genes involved in coenzyme Q synthesis during mouse ageing. MATERIAL AND METHODS: The messenger RNA (mRNA) of different organs, such as brain, liver, kidney and skeletal muscle from young (8 months), mature (18 months), and old (24 months) mice was extracted by using Trizol and was then analysed by real time PCR (qPCR) using specific primers for all the known components of the coenzyme Q-synthesis complex (COQ genes)...
July 20, 2017: Revista Española de Geriatría y Gerontología
https://www.readbyqxmd.com/read/28690551/impact-of-chemical-analogs-of-4-hydroxybenzoic-acid-on-coenzyme-q-biosynthesis-from-inhibition-to-bypass-of-coenzyme-q-deficiency
#5
REVIEW
Fabien Pierrel
Coenzyme Q is a lipid that participates to important physiological functions. Coenzyme Q is synthesized in multiple steps from the precursor 4-hydroxybenzoic acid. Mutations in enzymes that participate to coenzyme Q biosynthesis result in primary coenzyme Q deficiency, a type of mitochondrial disease. Coenzyme Q10 supplementation of patients is the classical treatment but it shows limited efficacy in some cases. The molecular understanding of the coenzyme Q biosynthetic pathway allowed the design of experiments to bypass deficient biosynthetic steps with analogs of 4-hydroxybenzoic acid...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28552678/detection-of-6-demethoxyubiquinone-in-coq10-deficiency-disorders-insights-into-enzyme-interactions-and-identification-of-potential-therapeutics
#6
Diran Herebian, Annette Seibt, Sander H J Smits, Gisela Bünning, Christoph Freyer, Holger Prokisch, Daniela Karall, Anna Wredenberg, Anna Wedell, Luis C López, Ertan Mayatepek, Felix Distelmaier
Coenzyme Q10 (CoQ10) is an essential cofactor of the mitochondrial oxidative phosphorylation (OXPHOS) system and its deficiency has important implications for several inherited metabolic disorders of childhood. The biosynthesis of CoQ10 is a complicated process, which involves at least 12 different enzymes. One of the metabolic intermediates that are formed during CoQ10 biosynthesis is the molecule 6-demethoxyubiquinone (6-DMQ). This CoQ precursor is processed at the level of COQ7 and COQ9. We selected this metabolite as a marker substance for metabolic analysis of cell lines with inherited genetic defects (COQ2, COQ4, COQ7 and COQ9) or siRNA knockdown in CoQ biosynthesis enzymes using ultra-performance liquid chromatography coupled to tandem mass spectrometry (UPLC-MS/MS)...
May 20, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28482003/coenzyme-q10-reduces-sevoflurane-induced-cognitive-deficiency-in-young-mice
#7
G Xu, H Lu, Y Dong, D Shapoval, S G Soriano, X Liu, Y Zhang, Z Xie
Background.: Anaesthesia can induce cognitive deficiency in young rodents and monkeys. Mitochondrial dysfunction contributes to the anaesthesia-induced neurotoxicity and neurobehavioural deficits. We therefore assessed the effects of the mitochondrial energy enhancer coenzyme Q 10 (CoQ 10 ) on anaesthesia-induced cognitive deficiency in young mice to investigate the role of mitochondrial dysfunction. Methods.: Young mice ( n =134) were randomly assigned into the following four groups: control plus corn oil vehicle (60% oxygen); 3% sevoflurane [2 h daily on postnatal day (P) 6, 7, and 8] plus vehicle; CoQ 10 (50 mg kg -1 ) plus vehicle; or 3% sevoflurane plus CoQ 10 plus vehicle...
May 7, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28472853/genetic-rescue-of-mitochondrial-and-skeletal-muscle-impairment-in-an-induced-pluripotent-stem-cells-model-of-coenzyme-q10-deficiency
#8
Damià Romero-Moya, Carlos Santos-Ocaña, Julio Castaño, Gloria Garrabou, José A Rodríguez-Gómez, Vanesa Ruiz-Bonilla, Clara Bueno, Patricia González-Rodríguez, Alessandra Giorgetti, Eusebio Perdiguero, Cristina Prieto, Constanza Moren-Nuñez, Daniel J Fernández-Ayala, Maria Victoria Cascajo, Iván Velasco, Josep Maria Canals, Raquel Montero, Delia Yubero, Cristina Jou, José López-Barneo, Francesc Cardellach, Pura Muñoz-Cánoves, Rafael Artuch, Plácido Navas, Pablo Menendez
Coenzyme Q10 (CoQ10 ) plays a crucial role in mitochondria as an electron carrier within the mitochondrial respiratory chain (MRC) and is an essential antioxidant. Mutations in genes responsible for CoQ10 biosynthesis (COQ genes) cause primary CoQ10 deficiency, a rare and heterogeneous mitochondrial disorder with no clear genotype-phenotype association, mainly affecting tissues with high-energy demand including brain and skeletal muscle (SkM). Here, we report a four-year-old girl diagnosed with minor mental retardation and lethal rhabdomyolysis harboring a heterozygous mutation (c...
July 2017: Stem Cells
https://www.readbyqxmd.com/read/28362733/phase-noise-characterization-of-sub-hertz-linewidth-lasers-via-digital-cross-correlation
#9
Xiaopeng Xie, Romain Bouchand, Daniele Nicolodi, Michel Lours, Christophe Alexandre, Yann Le Coq
Phase noise or frequency noise is a key metric to evaluate the short-term stability of a laser. This property is of great interest for the applications but delicate to characterize, especially for narrow linewidth lasers. In this Letter, we demonstrate a digital cross-correlation scheme to characterize the absolute phase noise of sub-hertz linewidth lasers. Three 1542 nm ultra-stable lasers are used in this approach. For each measurement, two lasers act as references to characterize a third one. Phase noise power spectral density from 0...
April 1, 2017: Optics Letters
https://www.readbyqxmd.com/read/28357388/balanced-coq6-biosynthesis-is-required-for-lifespan-and-mitophagy-in-yeast
#10
Isabel González-Mariscal, Aléjandro Martín-Montalvo, Cristina Ojeda-González, Adolfo Rodríguez-Eguren, Purificación Gutiérrez-Ríos, Plácido Navas, Carlos Santos-Ocaña
Coenzyme Q is an essential lipid with redox capacity that is present in all organisms. In yeast its biosynthesis depends on a multiprotein complex in which Coq7 protein has both catalytic and regulatory functions. Coq7 modulates CoQ6 levels through a phosphorylation cycle, where dephosphorylation of three amino acids (Ser/Thr) by the mitochondrial phosphatase Ptc7 increases the levels of CoQ6. Here we analyzed the role of Ptc7 and the phosphorylation state of Coq7 in yeast mitochondrial function. The conversion of the three Ser/Thr to alanine led to a permanently active form of Coq7 that caused a 2...
February 3, 2017: Microbial Cell
https://www.readbyqxmd.com/read/28339090/inhibition-of-farnesyl-pyrophosphate-synthase-attenuates-high-glucose%C3%A2-induced-vascular-smooth-muscle-cells-proliferation
#11
Guo-Ping Chen, Xiao-Qin Zhang, Tao Wu, Jie Han, Dan Ye
The proliferation of vascular smooth muscle cells (VSMCs) is one of the main features of atherosclerosis accelerated by hyperglycemia. Our previous studies found that farnesyl pyrophosphate synthase (FPPS, EC 2.5.1.10), an essential enzyme in the mevalonate pathway, was upregulated in aorta media from diabetic mice along with the process of atherosclerosis. However, the exact role of FPPS in high glucose‑induced proliferation of VSMCs is largely unclear. In our study, we found that alendronate (an FPPS inhibitor) attenuated diabetic accelerated atherosclerosis in vivo and suppressed high glucose‑induced VSMCs proliferation in vitro...
May 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28338638/the-value-of-coenzyme-q10-determination-in-mitochondrial-patients
#12
REVIEW
Delia Yubero, George Allen, Rafael Artuch, Raquel Montero
Coenzyme Q10 (CoQ) is a lipid that is ubiquitously synthesized in tissues and has a key role in mitochondrial oxidative phosphorylation. Its biochemical determination provides insight into the CoQ status of tissues and may detect CoQ deficiency that can result from either an inherited primary deficiency of CoQ metabolism or may be secondary to different genetic and environmental conditions. Rapid identification of CoQ deficiency can also allow potentially beneficial treatment to be initiated as early as possible...
March 24, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28329789/mediterranean-diet-supplemented-with-coenzyme-q10-modulates-the-postprandial-metabolism-of-advanced-glycation-end-products-in-elderly-men-and-women
#13
Javier Lopez-Moreno, Gracia M Quintana-Navarro, Javier Delgado-Lista, Antonio Garcia-Rios, Juan F Alcala-Diaz, Francisco Gomez-Delgado, Antonio Camargo, Pablo Perez-Martinez, Francisco J Tinahones, Gary E Striker, Francisco Perez-Jimenez, Jose M Villalba, Jose Lopez-Miranda, Elena M Yubero-Serrano
Advanced glycation end products (AGEs) and oxidative stress are elevated with aging and dysmetabolic conditions. Because a Mediterranean (Med) diet reduces oxidative stress, serum AGEs levels, and gene expression related to AGEs metabolism in healthy elderly people, we studied whether supplementation with coenzyme Q10 (CoQ) was of further benefit. Twenty participants aged ≥ 65 (10 men and 10 women) were randomly assigned to each of three isocaloric diets for successive periods of 4 weeks in a crossover design: Med diet, Med + CoQ, and a Western high-saturated-fat diet (SFA diet)...
October 21, 2016: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/28303649/transforming-sugars-into-fat-lipid-biosynthesis-using-different-sugars-in-yarrowia-lipolytica
#14
Piotr Hapeta, Magdalena Rakicka, Remi Dulermo, Heber Gamboa-Meléndez, Anne-Marie Cruz-Le Coq, Jean-Marc Nicaud, Zbigniew Lazar
In an era of ever-increasing energy demands, a promising technology is being developed: the use of oleaginous microorganisms such as Yarrowia lipolytica to convert waste materials into biofuels. Here, we constructed two Y. lipolytica strains that displayed both increased lipid accumulation and more efficient use of biomass-derived sugars, including glucose, fructose, galactose and inulin. The first strain, Y. lipolytica YLZ150, was derived from the French wild-type strain W29. It had inhibited triacylglycerol mobilization (∆tgl4) and β-oxidation (∆pox1-6), and it overexpressed GPD1, DGA2, HXK1, the native Leloir pathway, SUC2 from Saccharomyces cerevisiae and INU1 from Kluyveromyces marxianus...
March 17, 2017: Yeast
https://www.readbyqxmd.com/read/28272309/determination-of-the-clean-air-delivery-rate-cadr-of-photocatalytic-oxidation-pco-purifiers-for-indoor-air-pollutants-using-a-closed-loop-reactor-part-ii-experimental-results
#15
Valérie Héquet, Frédéric Batault, Cécile Raillard, Frédéric Thévenet, Laurence Le Coq, Éric Dumont
The performances of a laboratory PhotoCatalytic Oxidation (PCO) device were determined using a recirculation closed-loop pilot reactor. The closed-loop system was modeled by associating equations related to two ideal reactors: a perfectly mixed reservoir with a volume of VR = 0.42 m³ and a plug flow system corresponding to the PCO device with a volume of VP = 5.6 × 10(-3) m³. The PCO device was composed of a pleated photocatalytic filter (1100 cm²) and two 18-W UVA fluorescent tubes. The Clean Air Delivery Rate (CADR) of the apparatus was measured under different operating conditions...
March 6, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28205337/should-the-biofilm-mode-of-life-be-taken-into-consideration-for-microbial-biocontrol-agents
#16
REVIEW
Caroline Pandin, Dominique Le Coq, Alexis Canette, Stéphane Aymerich, Romain Briandet
Almost one-third of crop yields are lost every year due to microbial alterations and diseases. The main control strategy to limit these losses is the use of an array of chemicals active against spoilage and unwanted pathogenic microorganisms. Their massive use has led to extensive environmental pollution, human poisoning and a variety of diseases. An emerging alternative to this chemical approach is the use of microbial biocontrol agents. Biopesticides have been used with success in several fields, but a better understanding of their mode of action is necessary to better control their activity and increase their use...
July 2017: Microbial Biotechnology
https://www.readbyqxmd.com/read/28173653/-coenzyme-q-10-treatment-for-one-child-with-coq6-gene-mutation-induced-nephrotic-syndrome-and-literature-review
#17
REVIEW
Q Cao, G M Li, H Xu, Q Shen, L Sun, X Y Fang, H M Liu, W Guo, Y H Zhai, B B Wu
Objective: To summarize the clinical manifestation and molecular characteristics of COQ6 mutation induced nephrotic syndrome, and to evaluate efficacy of CoQ(10) therapy. Method: Clinical data of the case with infantile nephrotic syndrome was summarized, including clinical manifestations, laboratory findings and family investigation. The patient received CoQ(10) 30 mg/(kg·d) therapy. Urine protein/creatinine ratio, serum albumin and creatinine were detected to assess the efficacy of the therapy. Result: (1) The 10 months old boy was presented with nephrotic level proteinuria and hypoalbuminemia...
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28166796/coenzyme-q10-partially-restores-pathological-alterations-in-a-macrophage-model-of-gaucher-disease
#18
Mario de la Mata, David Cotán, Manuel Oropesa-Ávila, Marina Villanueva-Paz, Isabel de Lavera, Mónica Álvarez-Córdoba, Raquel Luzón-Hidalgo, Juan M Suárez-Rivero, Gustavo Tiscornia, José A Sánchez-Alcázar
BACKGROUND: Gaucher disease (GD) is caused by mutations in the GBA1 gene which encodes lysosomal β-glucocerebrosidase (GCase). In GD, partial or complete loss of GCase activity causes the accumulation of the glycolipids glucosylceramide (GlcCer) and glucosylsphingosine in the lysosomes of macrophages. In this manuscript, we investigated the effects of glycolipids accumulation on lysosomal and mitochondrial function, inflammasome activation and efferocytosis capacity in a THP-1 macrophage model of Gaucher disease...
February 6, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28107627/use-of-tissue-metabolite-analysis-and-enzyme-kinetics-to-discriminate-between-alternate-pathways-for-hydrogen-sulfide-metabolism
#19
Kristie D Cox Augustyn, Michael R Jackson, Marilyn Schuman Jorns
Hydrogen sulfide (H2S) is an endogenously synthesized signaling molecule that is enzymatically metabolized in mitochondria. The metabolism of H2S maintains optimal concentrations of the gasotransmitter and produces sulfane sulfur (S(0))-containing metabolites that may be functionally important in signaling. Sulfide:quinone oxidoreductase (SQOR) catalyzes the initial two-electron oxidation of H2S to S(0) using coenzyme Q as the electron acceptor in a reaction that requires a third substrate to act as the acceptor of S(0)...
February 21, 2017: Biochemistry
https://www.readbyqxmd.com/read/28099869/amitriptyline-down-regulates-coenzyme-q10-biosynthesis-in-lung-cancer-cells
#20
Tamara Ortiz, Marina Villanueva-Paz, Eduardo Díaz-Parrado, Matilde Illanes, Ana Fernández-Rodríguez, José A Sánchez-Alcázar, Manuel de Miguel
Amitriptyline, a tricyclic antidepressant, has been proposed as an antitumoral drug in oxidative therapy. Its pro-apoptotic effects, mediated by high reactive oxygen species generation, have been already described. In this study we analysed the effect of amitriptyline on the biosynthesis of coenzyme Q10 (CoQ), an essential component for electron transport and a potent membrane antioxidant involved in redox signaling. We treated H460 cells, a non-small-cell lung cancer cell line, with amitriptyline and we analysed CoQ levels by HPLC and CoQ biosynthesis rate, as well as the enzymes involved in CoQ biosynthesis by real-time PCR and Western blot...
February 15, 2017: European Journal of Pharmacology
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