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Coenzyme q

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https://www.readbyqxmd.com/read/29452608/insights-into-an-ancient-atypical-kinase-essential-for-biosynthesis-of-coenzyme-q
#1
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
#2
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
#3
Francisco M Gutierrez-Mariscal, Elena M Yubero-Serrano, Jose M Villalba, Jose Lopez-Miranda
Coenzyme Q 10 (CoQ 10 ) 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. CoQ 10 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 CoQ 10 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
#4
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
#5
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
#6
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-ducklings-skeletal-muscle-by-acting-on-the-substrate-oxidation-system
#7
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 gastrocnemius muscle of ducklings...
December 21, 2017: 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
#8
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...
January 12, 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
#9
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...
January 8, 2018: Journal of Biochemistry
https://www.readbyqxmd.com/read/29248374/multi-omics-reveal-specific-targets-of-the-rna-binding-protein-puf3p-and-its-orchestration-of-mitochondrial-biogenesis
#10
Christopher P Lapointe, Jonathan A Stefely, Adam Jochem, Paul D Hutchins, Gary M Wilson, Nicholas W Kwiecien, Joshua J Coon, Marvin Wickens, David J Pagliarini
Coenzyme Q (CoQ) is a redox-active lipid required for mitochondrial oxidative phosphorylation (OxPhos). How CoQ biosynthesis is coordinated with the biogenesis of OxPhos protein complexes is unclear. Here, we show that the Saccharomyces cerevisiae RNA-binding protein (RBP) Puf3p regulates CoQ biosynthesis. To establish the mechanism for this regulation, we employed a multi-omic strategy to identify mRNAs that not only bind Puf3p but also are regulated by Puf3p in vivo. The CoQ biosynthesis enzyme Coq5p is a critical Puf3p target: Puf3p regulates the abundance of Coq5p and prevents its detrimental hyperaccumulation, thereby enabling efficient CoQ production...
December 12, 2017: Cell Systems
https://www.readbyqxmd.com/read/29220658/multi-omic-mitoprotease-profiling-defines-a-role-for-oct1p-in-coenzyme-q-production
#11
Mike T Veling, Andrew G Reidenbach, Elyse C Freiberger, Nicholas W Kwiecien, Paul D Hutchins, Michael J Drahnak, Adam Jochem, Arne Ulbrich, Matthew J P Rush, Jason D Russell, Joshua J Coon, David J Pagliarini
Mitoproteases are becoming recognized as key regulators of diverse mitochondrial functions, although their direct substrates are often difficult to discern. Through multi-omic profiling of diverse Saccharomyces cerevisiae mitoprotease deletion strains, we predicted numerous associations between mitoproteases and distinct mitochondrial processes. These include a strong association between the mitochondrial matrix octapeptidase Oct1p and coenzyme Q (CoQ) biosynthesis-a pathway essential for mitochondrial respiration...
December 7, 2017: Molecular Cell
https://www.readbyqxmd.com/read/29211771/short-term-succinic-acid-treatment-mitigates-cerebellar-mitochondrial-oxphos-dysfunction-neurodegeneration-and-ataxia-in-a-purkinje-specific-spinocerebellar-ataxia-type-1-sca1-mouse-model
#12
Austin Ferro, Emily Carbone, Jenny Zhang, Evan Marzouk, Monica Villegas, Asher Siegel, Donna Nguyen, Thomas Possidente, Jessilyn Hartman, Kailen Polley, Melissa A Ingram, Georgia Berry, Thomas H Reynolds, Bernard Possidente, Kimberley Frederick, Stephen Ives, Sarita Lagalwar
Mitochondrial dysfunction plays a significant role in neurodegenerative disease including ataxias and other movement disorders, particularly those marked by progressive degeneration in the cerebellum. In this study, we investigate the role of mitochondrial oxidative phosphorylation (OXPHOS) deficits in cerebellar tissue of a Purkinje cell-driven spinocerebellar ataxia type 1 (SCA1) mouse. Using RNA sequencing transcriptomics, OXPHOS complex assembly analysis and oxygen consumption assays, we report that in the presence of mutant polyglutamine-expanded ataxin-1, SCA1 mice display deficits in cerebellar OXPHOS complex I (NADH-coenzyme Q oxidoreductase)...
2017: PloS One
https://www.readbyqxmd.com/read/29204204/thiamine-responsive-pyruvate-dehydrogenase-complex-deficiency-a-potentially-treatable-cause-of-leigh-s-disease
#13
Prashant Jauhari, Naveen Sankhyan, Sameer Vyas, Pratibha Singhi
Pyruvate dehydrogenase complex (PDHC) deficiency is a rare metabolic disorder that affects tissues with high energy demand such as the central nervous system. The clinico-radiological phenotype of Leigh's disease is one of its common presentations. We present a 9-month-old boy with rapidly progressive infantile Leigh's disease. PDHA1 gene sequencing revealed a pathological homozygous missense mutation c.131A>G or p.H44R in exon 3 consistent with PDHC deficiency. H44R is among the five mutations (H44R, R88S, G89S, R263G, and V389fs) in E1α subunit that is thiamine-responsive...
July 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29198567/conserved-lipid-and-small-molecule-modulation-of-coq8-reveals-regulation-of-the-ancient-kinase-like-ubib-family
#14
Andrew G Reidenbach, Zachary A Kemmerer, Deniz Aydin, Adam Jochem, Molly T McDevitt, Paul D Hutchins, Jaime L Stark, Jonathan A Stefely, Thiru Reddy, Alex S Hebert, Emily M Wilkerson, Isabel E Johnson, Craig A Bingman, John L Markley, Joshua J Coon, Matteo Dal Peraro, David J Pagliarini
Human COQ8A (ADCK3) and Saccharomyces cerevisiae Coq8p (collectively COQ8) are UbiB family proteins essential for mitochondrial coenzyme Q (CoQ) biosynthesis. However, the biochemical activity of COQ8 and its direct role in CoQ production remain unclear, in part due to lack of known endogenous regulators of COQ8 function and of effective small molecules for probing its activity in vivo. Here, we demonstrate that COQ8 possesses evolutionarily conserved ATPase activity that is activated by binding to membranes containing cardiolipin and by phenolic compounds that resemble CoQ pathway intermediates...
November 24, 2017: Cell Chemical Biology
https://www.readbyqxmd.com/read/29194833/mutations-in-coq8b-adck4-found-in-patients-with-steroid-resistant-nephrotic-syndrome-alter-coq8b-function
#15
Luis Vazquez Fonseca, Mara Doimo, Cristina Calderan, Maria Andrea Desbats, Manuel J Acosta, Cristina Cerqua, Matteo Cassina, Shazia Ashraf, Friedhelm Hildebrandt, Geppo Sartori, Placido Navas, Eva Trevisson, Leonardo Salviati
Mutations in COQ8B cause steroid-resistant nephrotic syndrome with variable neurological involvement. In yeast, COQ8 encodes a protein required for coenzyme Q (CoQ) biosynthesis, whose precise role is not clear. Humans harbor two paralog genes: COQ8A and COQ8B (previously termed ADCK3 and ADCK4). We have found that COQ8B is a mitochondrial matrix protein peripherally associated with the inner membrane. COQ8B can complement a ΔCOQ8 yeast strain when its mitochondrial targeting sequence (MTS) is replaced by a yeast MTS...
December 1, 2017: Human Mutation
https://www.readbyqxmd.com/read/29191091/schizosaccharomyces-japonicus-has-low-levels-of-coq10-synthesis-respiration-deficiency-and-efficient-ethanol-production
#16
Tomohiro Kaino, Kai Tonoko, Shiomi Mochizuki, Yuriko Takashima, Makoto Kawamukai
Coenzyme Q (CoQ) is essential for mitochondrial respiration and as a cofactor for sulfide quinone reductase. Schizosaccharomyces pombe produces a human-type CoQ10. Here, we analyzed CoQ in other fission yeast species. S. cryophilus and S. octosporus produce CoQ9. S. japonicus produces low levels of CoQ10, although all necessary genes for CoQ synthesis have been identified in its genome. We expressed three genes (dps1, dlp1, and ppt1) for CoQ synthesis from S. japonicus in the corresponding S. pombe mutants, and confirmed that they were functional...
December 1, 2017: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/29190290/skeletal-muscle-mitochondrial-bioenergetics-and-associations-with-myostatin-genotypes-in-the-thoroughbred-horse
#17
Mary F Rooney, Richard K Porter, Lisa M Katz, Emmeline W Hill
Variation in the myostatin (MSTN) gene has been reported to be associated with race distance, body composition and skeletal muscle fibre composition in the horse. The aim of the present study was to test the hypothesis that MSTN variation influences mitochondrial phenotypes in equine skeletal muscle. Mitochondrial abundance and skeletal muscle fibre types were measured in whole muscle biopsies from the gluteus medius of n = 82 untrained (21 ± 3 months) Thoroughbred horses. Skeletal muscle fibre type proportions were significantly (p < 0...
2017: PloS One
https://www.readbyqxmd.com/read/29159460/compound-heterozygous-inheritance-of-mutations-in-coenzyme-q8a-results-in-autosomal-recessive-cerebellar-ataxia-and-coenzyme-q10-deficiency-in-a-female-sib-pair
#18
Jessie C Jacobsen, Whitney Whitford, Brendan Swan, Juliet Taylor, Donald R Love, Rosamund Hill, Sarah Molyneux, Peter M George, Richard Mackay, Stephen P Robertson, Russell G Snell, Klaus Lehnert
Autosomal recessive ataxias are characterised by a fundamental loss in coordination of gait with associated atrophy of the cerebellum. There is significant clinical and genetic heterogeneity amongst inherited ataxias; however, an early molecular diagnosis is essential with low-risk treatments available for some of these conditions. We describe two female siblings who presented early in life with unsteady gait and cerebellar atrophy. Whole exome sequencing revealed compound heterozygous inheritance of two pathogenic mutations (p...
November 21, 2017: JIMD Reports
https://www.readbyqxmd.com/read/29137472/changes-in-transcription-pattern-lead-to-a-marked-decrease-in-cox-cs-and-sqr-activity-after-the-developmental-point-of-the-22-nd-gestational-week
#19
H Kolarova, J Krizova, M Hulkova, H Hansikova, H Hulkova, V Smid, J Zeman, T Honzik, M Tesarova
Tissue differentiation and proliferation throughout fetal development interconnect with changes in the Oxidative Phosphorylation System (OXPHOS) on the cellular level. Reevaluation of the expression data revealed a significant increase in COX4 and MTATP6 liver transcription levels after the 22(nd) gestational week (GW) which inspired us to characterize its functional impact. Specific activities of cytochrome c oxidase (COX), citrate synthase (CS), succinate-coenzyme Q reductase (SQR) and mtDNA determined by spectrophotometry and RT-PCR were studied in a set of 25 liver and 18 skeletal muscle samples at 13(th) to 29(th) GW...
November 10, 2017: Physiological Research
https://www.readbyqxmd.com/read/29132502/transcriptomic-and-proteomic-landscape-of-mitochondrial-dysfunction-reveals-secondary-coenzyme-q-deficiency-in-mammals
#20
Inge Kühl, Maria Miranda, Ilian Atanassov, Irina Kuznetsova, Yvonne Hinze, Arnaud Mourier, Aleksandra Filipovska, Nils-Göran Larsson
Dysfunction of the oxidative phosphorylation (OXPHOS) system is a major cause of human disease and the cellular consequences are highly complex. Here, we present comparative analyses of mitochondrial proteomes, cellular transcriptomes and targeted metabolomics of five knockout mouse strains deficient in essential factors required for mitochondrial DNA gene expression, leading to OXPHOS dysfunction. Moreover, we describe sequential protein changes during post-natal development and progressive OXPHOS dysfunction in time course analyses in control mice and a middle lifespan knockout, respectively...
November 14, 2017: ELife
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