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Coenzyme q

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https://www.readbyqxmd.com/read/29220658/multi-omic-mitoprotease-profiling-defines-a-role-for-oct1p-in-coenzyme-q-production
#1
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
#2
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
#3
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
#4
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
#5
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 paralogue 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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/29082420/substrate-specificity-and-membrane-topologies-of-the-iron-containing-%C3%AF-3-and-%C3%AF-6-desaturases-from-mortierella-alpina
#11
Mingxuan Wang, Haiqin Chen, Aisikaer Ailati, Wei Chen, Floyd H Chilton, W Todd Lowther, Yong Q Chen
Polyunsaturated fatty acids (PUFAs) are essential lipids for cell function, normal growth, and development, serving as key structural components of biological membranes and modulating critical signal transduction events. Omega-3 (n-3) long chain PUFAs (LC-PUFAs) such as eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) have been shown to protect against inflammatory diseases and enhance brain development and function. This had led to a marked increase in demand for fish and fish oils in human diets, supplements, and aquaculture and created a need for new, sustainable n-3 LC-PUFA sources...
October 30, 2017: Applied Microbiology and Biotechnology
https://www.readbyqxmd.com/read/29025968/activation-of-ampk-mtorc1-mediated-autophagy-by-metformin-reverses-clk1-deficiency-sensitized-dopaminergic-neuronal-death
#12
Qiuting Yan, Chaojun Han, Guanghui Wang, John L Waddington, Longtai Zheng, Xuechu Zhen
The autophagy-lysosome pathway (ALP) plays a critical role in the pathology of Parkinson's disease (PD). Clk1 (coq7) is a mitochondrial hydroxylase that is essential for coenzyme Q (ubiquinone) biosynthesis. We have reported previously that Clk1 regulates microglia activation via modulating microglia metabolic reprogramming, which contributes to dopaminergic neuronal survival. This study explores the direct effect of Clk1 on dopaminergic neuronal survival. We demonstrate that Clk1 deficiency inhibited dopaminergic neuronal autophagy in cultured MN9D dopaminergic neurons and in the substantia nigra pars compacta of Clk+/- mutant mice and consequently sensitized dopaminergic neuron damage and behavioral defects...
December 2017: Molecular Pharmacology
https://www.readbyqxmd.com/read/29020939/a-transcriptomic-approach-to-study-the-effect-of-long-term-starvation-and-diet-composition-on-the-expression-of-mitochondrial-oxidative-phosphorylation-genes-in-gilthead-sea-bream-sparus-aurata
#13
Jonás I Silva-Marrero, Alberto Sáez, Albert Caballero-Solares, Ivan Viegas, María Pilar Almajano, Felipe Fernández, Isabel V Baanante, Isidoro Metón
BACKGROUND: The impact of nutritional status and diet composition on mitochondrial oxidative phosphorylation (OXPHOS) in fish remains largely unknown. To identify biomarkers of interest in nutritional studies, herein we obtained a deep-coverage transcriptome by 454 pyrosequencing of liver and skeletal muscle cDNA normalised libraries from long-term starved gilthead sea bream (Sparus aurata) and fish fed different diets. RESULTS: After clean-up of high-throughput deep sequencing reads, 699,991 and 555,031 high-quality reads allowed de novo assembly of liver and skeletal muscle sequences, respectively (average length: 374 and 441 bp; total megabases: 262 and 245 Mbp)...
October 11, 2017: BMC Genomics
https://www.readbyqxmd.com/read/28989672/photochromic-coenzyme-q-derivatives-switching-redox-potentials-with-light
#14
Nadja A Simeth, Andrea C Kneuttinger, Reinhard Sterner, Burkhard König
Coenzyme Q is an important redox cofactor involved in a variety of cellular processes, and is thus found in several cell compartments. We report a photochromic derivative of coenzyme Q that combines the molecular structures of the redox active cofactor and a photochromic dye. Light irradiation triggers an electronic rearrangement reversibly changing the redox potential. We used this effect to control the intermolecular redox reaction of the photochromic coenzyme Q derivative with dihydropyridine in solution by light irradiation...
September 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/28927698/biochemistry-of-mitochondrial-coenzyme-q-biosynthesis
#15
REVIEW
Jonathan A Stefely, David J Pagliarini
Coenzyme Q (CoQ, ubiquinone) is a redox-active lipid produced across all domains of life that functions in electron transport and oxidative phosphorylation and whose deficiency causes human diseases. Yet, CoQ biosynthesis has not been fully defined in any organism. Several proteins with unclear molecular functions facilitate CoQ biosynthesis through unknown means, and multiple steps in the pathway are catalyzed by currently unidentified enzymes. Here we highlight recent progress toward filling these knowledge gaps through both traditional biochemistry and cutting-edge 'omics' approaches...
October 2017: Trends in Biochemical Sciences
https://www.readbyqxmd.com/read/28925477/the-study-on-the-effect-of-hyperglycemia-on-offspring-fatty-tissue-metabolism-during-pregnancy
#16
H-P Dong, M-Z Tan, Q-J Liu, J Wang, S-B Zhong
OBJECTIVE: In this study, we adopted maternal diabetes model on rats, which induced by streptozotocin to explore the metabolism changes of rat adipose tissue during the neonatal period. MATERIALS AND METHODS: The female rats were induced as diabetes models by streptozotocin (STZ), and mated with the normal male rats when they entered into adulthood. The chosen male offspring rats were executed at week 12 and the epididymis and subcutaneous fat pad were obtained...
August 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28918503/coq0-induced-mitochondrial-ptp-opening-triggers-apoptosis-via-ros-mediated-vdac1-upregulation-in-hl-60-leukemia-cells-and-suppresses-tumor-growth-in-athymic-nude-mice-xenografted-nude-mice
#17
You-Cheng Hseu, Varadharajan Thiyagarajan, Ting-Tsz Ou, Hsin-Ling Yang
Coenzyme Q (CoQ) analogs with variable numbers of isoprenoid units have been demonstrated as anticancer and antioxidant/pro-oxidant molecules. This study examined the in vitro and in vivo antitumor and apoptosis activities of CoQ0 (2,3-dimethoxy-5-methyl-1,4-benzoquinone, zero isoprenoid side-chains) through upregulation of the Voltage-dependent anion channel 1 (VDAC1) signaling pathway on human promyelocytic leukemia. CoQ0 (0-40 μg/mL) treatment significantly reduced HL-60 cell viability, and up-regulated mitochondrial VDAC1 expression...
September 16, 2017: Archives of Toxicology
https://www.readbyqxmd.com/read/28915357/a-yigp-mutant-strain-is-a-small-colony-variant-of-e-coli-and-shows-pleiotropic-antibiotic-resistance
#18
Hui Xia, Qiongwei Tang, Jie Song, Jiang Ye, Haizhen Wu, Huizhan Zhang
Small colony variants (SCVs) are a commonly observed subpopulation of bacteria that have a small colony size and distinctive biochemical characteristics. SCVs are more resistant than the wild type to some antibiotics and usually cause persistent infections in the clinic. SCV studies have been very active during the past 2 decades, especially Staphylococcus aureus SCVs. However, fewer studies on Escherichia coli SCVs exist, so we studied an E. coli SCV during an experiment involving the deletion of the yigP locus...
September 15, 2017: Canadian Journal of Microbiology
https://www.readbyqxmd.com/read/28900834/coq10-increases-mitochondrial-mass-and-polarization-atp-and-oct4-potency-levels-and-bovine-oocyte-mii-during-ivm-while-decreasing-ampk-activity-and-oocyte-death
#19
M K Abdulhasan, Q Li, J Dai, H M Abu-Soud, E E Puscheck, D A Rappolee
PURPOSE: We tested whether mitochondrial electron transport chain electron carrier coenzyme Q10 (CoQ10) increases ATP during bovine IVM and increases %M2 oocytes, mitochondrial polarization/mass, and Oct4, and decreases pAMPK and oocyte death. METHODS: Bovine oocytes were aspirated from ovaries and cultured in IVM media for 24 h with 0, 20, 40, or 60 μM CoQ10. Oocytes were assayed for ATP by luciferase-based luminescence. Oocyte micrographs were quantitated for Oct4, pAMPK (i...
September 12, 2017: Journal of Assisted Reproduction and Genetics
https://www.readbyqxmd.com/read/28890359/optic-atrophy-1-mediates-coenzyme-q-responsive-regulation-of-respiratory-complex-iv-activity-in-brain-mitochondria
#20
Kazuhide Takahashi, Ikuroh Ohsawa, Takuji Shirasawa, Mayumi Takahashi
The oxygen consumption rate (OCR) in brain mitochondria is significantly lower in aged mice than in young mice, and the reduced OCR is rescued by administration of water-solubilized CoQ10 to aged mice via drinking water. However, the mechanism behind this remains unclear. Here, we show that the activity of respiratory complex IV (CIV) in brain mitochondria declined in aged mice than in young mice, with no significant change in individual respiratory complex levels and their supercomplex assembly. Reduced CIV activity in the aged mice coincided with reduced binding of optic atrophy 1 (OPA1) to CIV...
September 7, 2017: Experimental Gerontology
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