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Coenzyme q

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https://www.readbyqxmd.com/read/28515908/renal-manifestations-of-primary-mitochondrial-disorders
#1
Josef Finsterer, Fulvio Scorza
The aim of the present review was to summarize and discuss previous findings concerning renal manifestations of primary mitochondrial disorders (MIDs). A literature review was performed using frequently used databases. The study identified that primary MIDs frequently present as mitochondrial multiorgan disorder syndrome (MIMODS) at onset or in the later course of the MID. Occasionally, the kidneys are affected in MIDs. Renal manifestations of MIDs include renal insufficiency, nephrolithiasis, nephrotic syndrome, renal cysts, renal tubular acidosis, Bartter-like syndrome, Fanconi syndrome, focal segmental glomerulosclerosis, tubulointerstitial nephritis, nephrocalcinosis, and benign or malign neoplasms...
May 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28512131/h2s-oxidation-by-nanodisc-embedded-human-sulfide-quinone-oxidoreductase
#2
Aaron P Landry, David P Ballou, Ruma Banerjee
Buildup of hydrogen sulfide (H2S), which functions as a signaling molecule but is toxic at high concentrations, is averted by its efficient oxidation by the mitochondrial sulfide oxidation pathway. The first step in this pathway is catalyzed by a flavoprotein, sulfide quinone oxidoreductase (SQR), which converts H2S to a persulfide and transfers electrons to coenzyme Q via a flavin cofactor. All previous studies on human SQR have used detergent-solubilized protein. Here, we embedded human SQR in nanodiscs (ndSQR) and studied highly homogenous preparations by steady-state and rapid kinetics techniques...
May 16, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28482003/coenzyme-q10-reduces-sevoflurane-induced-cognitive-deficiency-in-young-mice
#3
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/28480405/potential-administration-of-lipoic-acid-and-coenzyme-q-against-adipogensis-target-for-weight-reduction
#4
Maryam A Al-Ghamdi, Hani Choudhry, Huda A Al-Doghather, Etimad H Huwait, Taha A Kumosani, Said S Moselhy
BACKGROUND: Body overweight and obesity were considered as a risk factor for many systemic diseases as diabetic hypertension, cardiovascular diseases, and some cancers. The lipoic acid and Co Q are considered as coenzymes needed for enhancement metabolic rate. The goal of this study is to evaluate the anti-obese effect of lipoic acid alone or combined with Co-Q in rats. MATERIALS AND METHODS: Ninety male albino rats (100-150g) were used in this study, divided into six groups (15 each)...
2017: African Journal of Traditional, Complementary, and Alternative Medicines: AJTCAM
https://www.readbyqxmd.com/read/28380598/dietary-whole-and-cracked-linseed-increases-the-proportion-of-oleic-and-%C3%AE-linolenic-acids-in-adipose-tissues-and-decreases-stearoyl-coenzyme-a-desaturase-acetyl-coenzyme-a-carboxylase-and-fatty-acid-synthase-gene-expression-in-the-longissimus-thoracis-muscle
#5
X Z Li, C G Yan, J Yu, Q S Gao, S H Choi, J S Shin, S B Smith
We hypothesized that supplementation of linseed in a beef cattle fattening diet would increase PUFA concentrations in intramuscular adipose tissue and depress (), (), and () gene expression by decreasing () expression. Conversely, supplemental linseed would upregulate expression of () and () in muscle of Yanbian Yellow steers. Thirty steers were assigned at random to 3 groups of 10 steers fed either the basal diet (corn grain and corn silage-based commercial concentrate [CON]), the CON diet plus 8% whole linseed (WLS; DM basis), or the CON diet plus 8% cracked linseed (CLS; DM basis) for 6 mo...
February 2017: Journal of Animal Science
https://www.readbyqxmd.com/read/28370744/novel-mutations-and-expression-changes-of-acetyl-coenzyme-a-carboxylase-are-associated-with-spirotetramat-resistance-in-aphis-gossypii-glover
#6
Y Pan, E Zhu, X Gao, R Nauen, J Xi, T Peng, X Wei, C Zheng, Q Shang
Acetyl-coenzyme A carboxylase (ACC) catalyses the carboxylation of acetyl-coenzyme A (acetyl-CoA) to produce malonyl-CoA during the de novo synthesis of fatty acids. Spirotetramat, an inhibitor of ACC, is widely used to control a range of sucking insects, including the Aphis gossypii. In the present study, Reverse transcription quantitative real-time PCR (RT-qPCR) results demonstrated that ACC was significantly overexpressed in a laboratory-selected spirotetramat-resistant strain compared with the susceptible strain...
March 31, 2017: Insect Molecular Biology
https://www.readbyqxmd.com/read/28357388/balanced-coq6-biosynthesis-is-required-for-lifespan-and-mitophagy-in-yeast
#7
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/28357127/metabolic-effects-of-a-mitochondrial-targeted-coenzyme-q-analog-in-high-fat-fed-obese-mice
#8
Brian D Fink, Deng Fu Guo, Chaitanya A Kulkarni, Kamal Rahmouni, Robert J Kerns, William I Sivitz
We recently reported that mitoquinone (mitoQ, 500 μmol/L) added to drinking water of C57BL/6J mice attenuated weight gain, decreased food intake, increased hypothalamic orexigenic gene expression, and mitigated oxidative stress when administered from the onset of high-fat (HF) feeding. Here, we examined the effects of mitoQ on pre-existing obesity in C57BL/6J mice first made obese by 107 days of HF feeding. In contrast to our preventative study, we found that already obese mice did not tolerate mitoQ at 500 μmol/L...
April 2017: Pharmacology Research & Perspectives
https://www.readbyqxmd.com/read/28314870/perillyl-alcohol-a-pleiotropic-natural-compound-suitable-for-brain-tumor-therapy-targets-free-radicals
#9
REVIEW
Aline C Gomes, Angélica L Mello, Manuel G Ribeiro, Diogo G Garcia, Clovis O Da Fonseca, Marcela D'Alincourt Salazar, Axel H Schönthal, Thereza Quirico-Santos
Monoterpenes such as limonene and perillyl alcohol (POH) are promising natural compounds with pro-oxidant properties partly due to endoplasmic reticulum (ER) stress-induced cytotoxicity, and antioxidant activity owing to their activity as free radical scavengers, inhibition of coenzyme Q synthesis, activation of antioxidant-responsive elements (inducing detoxification enzymes) and induction of apoptosis. Activation of ER-stress responses generates reactive oxygen species (ROS), which are highly reactive free radicals mainly produced during mitochondrial electron transfer for adenosine triphosphate (ATP) synthesis...
March 17, 2017: Archivum Immunologiae et Therapiae Experimentalis
https://www.readbyqxmd.com/read/28173653/-coenzyme-q-10-treatment-for-one-child-with-coq6-gene-mutation-induced-nephrotic-syndrome-and-literature-review
#10
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/28138430/nd2-mutation-with-minimal-coenzyme-q-responsive-manifestations
#11
Josef Finsterer, Sinda Zarrouk-Mahjoub
No abstract text is available yet for this article.
March 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28107627/use-of-tissue-metabolite-analysis-and-enzyme-kinetics-to-discriminate-between-alternate-pathways-for-hydrogen-sulfide-metabolism
#12
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/28047568/we-fg-bra-12-research-work-of-the-radio-dynamic-treatment-mechanism
#13
Quanshi Zhang, Q Y Sun, G P Xiao, J Zeng, L Wang, L L Chen, C M C Ma
PURPOSE: The finite penetration depth of Laser light has limited clinical applications for PDT. This present work investigates the activation of photosensitizers using Cerenkov light emission from 45MV photon beams produced in an LA45 cancer therapy accelerator. We have named this new treatment technique Radio-Dynamic Therapy (RDT). METHODS: Monte Carlo simulations were made on various Cerenkov emission energies and their spectroscopy in excited target areas in order to estimate their photosensitizer inner activation efficiency...
June 2016: Medical Physics
https://www.readbyqxmd.com/read/28001060/biosynthesis-of-antroquinonol-and-4-acetylantroquinonol-b-via-a-polyketide-pathway-using-orsellinic-acid-as-a-ring-precursor-in-antrodia-cinnamomea
#14
Kevin Chi-Chung Chou, Shang-Han Yang, Hsiang-Lin Wu, Pei-Yin Lin, Tsu-Liang Chang, Fuu Sheu, Kai-Hsien Chen, Been-Huang Chiang
Antroquinonol (AQ) and 4-acetylantroquinonol B (4-AAQB), isolated from the mycelium of Antrodia cinnamomea, have a similar chemical backbone to coenzyme Q (CoQ). Based on the postulation that biosynthesis of both AQ and 4-AAQB in A. cinnamomea starts from the polyketide pathway, we cultivated this fungus in a culture medium containing [U-(13)C]oleic acid, and then we analyzed the crude extracts of the mycelium using UHPLC-MS. We found that AQ and 4-AAQB follow similar biosynthetic sequences as CoQ. Obvious [(13)C2] fragments on the ring backbone were detected in the mass spectrum for [(13)C2]AQ, [(13)C2]4-AAQB, and their [(13)C2] intermediates found in this study...
January 11, 2017: Journal of Agricultural and Food Chemistry
https://www.readbyqxmd.com/read/27938594/-clinical-biochemical-and-gene-mutation-characteristics-of-short-chain-acyl-coenzyme-a-dehydrogenase-deficiency-by-neonatal-screening
#15
X W Huang, Y Zhang, J B Yang, F Hong, G L Qian, F Tong, H Q Mao, X L Huang, X L Zhou, R L Yang, Z Y Zhao
Objective: To investigate the incidence, clinical, biochemical and gene mutation characteristics of short chain acyl-coenzyme A dehydrogenase deficiency (SCADD). Method: From January, 2009 to October, 2015, a retrospective analysis of the urine organic acids and acyl-coenzyme A dehydrogenase (ACADS) gene mutation characteristics of patients diagnosed as SCADD by newborn screening using tandem mass spectrometry in Department of Genetics and Metabolism (Newborn screening Center of Zhejiang Province), Children's Hospital, Zhejiang University School of Medicine...
December 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27909452/the-levels-of-cortisol-and-oxidative-stress-and-dna-damage-in-child-and-adolescent-victims-of-sexual-abuse-with-or-without-post-traumatic-stress-disorder
#16
Şeref Şimşek, Tuğba Yüksel, İbrahim Kaplan, Cem Uysal, Hüseyin Aktaş
OBJECTIVE: The aim of this study was to investigate whether cortisol and oxidative stress levels and DNA damage differ between individuals who developed PTSD or not following a sexual trauma. METHODS: The study included 61 children aged between 5 and 17 years who sustained sexual abuse (M/F: 18/43). The patients were divided into two groups: patients with PTSD and patients without PTSD based, based on the results of a structured psychiatric interview (K-SADS-PL and CAPS-CA)...
November 2016: Psychiatry Investigation
https://www.readbyqxmd.com/read/27897404/doxorubicin-inhibits-muscle-inflammation-after-eccentric-exercise
#17
Sheng-Chih Huang, Jin-Fu Wu, Suchada Saovieng, Wei-Horng Chien, Ming-Fen Hsu, Xiao-Fei Li, Shin-Da Lee, Chih-Yang Huang, Chih-Yang Huang, Chia-Hua Kuo
BACKGROUND: Doxorubicin, a widely used anti-tumour drug, is known to cause muscle loss in cancer patients. METHODS: Following an acute dose of doxorubicin injection (2.5 mg/kg per body weight), we examined macrophage distribution in rat soleus muscle challenged by eccentric exercise (downhill running). Long-term doxorubicin treatment (one injection every 3 days) on muscle mass and survival were also determined. RESULTS: Under non-exercised condition, increased tumour necrosis factor (TNF)-alpha mRNA and decreased IL-10 mRNA were observed in soleus muscle of doxorubicin-treated rats, compared with saline-treated control rats...
April 2017: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/27872580/untargeted-polar-metabolomics-of-transformed-mda-mb-231-breast-cancer-cells-expressing-varying-levels-of-human-arylamine-n-acetyltransferase-1
#18
Samantha M Carlisle, Patrick J Trainor, Xinmin Yin, Mark A Doll, Marcus W Stepp, J Christopher States, Xiang Zhang, David W Hein
INTRODUCTION: Human arylamine N-acetyltransferase 1 (NAT1) is a phase II xenobiotic metabolizing enzyme found in almost all tissues. Expression of NAT1 is elevated in several cancers including breast cancer. However, the exact mechanism by which NAT1 expression affects cancer risk and progression remains unclear. OBJECTIVE: This study explored polar metabolome differences between MDA-MB-231 breast cancer cells expressing varying levels of NAT1 activity using an untargeted approach...
July 2016: Metabolomics: Official Journal of the Metabolomic Society
https://www.readbyqxmd.com/read/27856619/coq-deficiency-causes-disruption-of-mitochondrial-sulfide-oxidation-a-new-pathomechanism-associated-with-this-syndrome
#19
Marta Luna-Sánchez, Agustín Hidalgo-Gutiérrez, Tatjana M Hildebrandt, Julio Chaves-Serrano, Eliana Barriocanal-Casado, Ángela Santos-Fandila, Miguel Romero, Ramy Ka Sayed, Juan Duarte, Holger Prokisch, Markus Schuelke, Felix Distelmaier, Germaine Escames, Darío Acuña-Castroviejo, Luis C López
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain, but it also has several other functions in the cellular metabolism. One of them is to function as an electron carrier in the reaction catalyzed by sulfide:quinone oxidoreductase (SQR), which catalyzes the first reaction in the hydrogen sulfide oxidation pathway. Therefore, SQR may be affected by CoQ deficiency. Using human skin fibroblasts and two mouse models with primary CoQ deficiency, we demonstrate that severe CoQ deficiency causes a reduction in SQR levels and activity, which leads to an alteration of mitochondrial sulfide metabolism...
January 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/27856618/coenzyme-q-deficiency-causes-impairment-of-the-sulfide-oxidation-pathway
#20
Marcello Ziosi, Ivano Di Meo, Giulio Kleiner, Xing-Huang Gao, Emanuele Barca, Maria J Sanchez-Quintero, Saba Tadesse, Hongfeng Jiang, Changhong Qiao, Richard J Rodenburg, Emmanuel Scalais, Markus Schuelke, Belinda Willard, Maria Hatzoglou, Valeria Tiranti, Catarina M Quinzii
Coenzyme Q (CoQ) is an electron acceptor for sulfide-quinone reductase (SQR), the first enzyme of the hydrogen sulfide oxidation pathway. Here, we show that lack of CoQ in human skin fibroblasts causes impairment of hydrogen sulfide oxidation, proportional to the residual levels of CoQ. Biochemical and molecular abnormalities are rescued by CoQ supplementation in vitro and recapitulated by pharmacological inhibition of CoQ biosynthesis in skin fibroblasts and ADCK3 depletion in HeLa cells. Kidneys of Pdss2(kd/kd) mice, which only have ~15% residual CoQ concentrations and are clinically affected, showed (i) reduced protein levels of SQR and downstream enzymes, (ii) accumulation of hydrogen sulfides, and (iii) glutathione depletion...
January 2017: EMBO Molecular Medicine
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