keyword
MENU ▼
Read by QxMD icon Read
search

Mitochondria AND CoQ10

keyword
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
#1
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...
May 4, 2017: Stem Cells
https://www.readbyqxmd.com/read/28428331/a-personalized-model-of-coq2-nephropathy-rescued-by-the-wild-type-coq2-allele-or-dietary-coenzyme-q10-supplementation
#2
Jun-Yi Zhu, Yulong Fu, Adam Richman, Zhanzheng Zhao, Patricio E Ray, Zhe Han
Clinical studies have identified patients with nephrotic syndrome caused by mutations in genes involved in the biosynthesis of coenzyme Q10 (CoQ10), a lipid component of the mitochondrial electron transport chain and an important antioxidant. However, the cellular mechanisms through which these mutations induce podocyte injury remain obscure. Here, we exploited the striking similarities between Drosophila nephrocytes and human podocytes to develop a Drosophila model of these renal diseases, and performed a systematic in vivo analysis assessing the role of CoQ10 pathway genes in renal function...
April 20, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28424622/pravastatin-chronic-treatment-sensitizes-hypercholesterolemic-mice-muscle-to-mitochondrial-permeability-transition-protection-by-creatine-or-coenzyme-q10
#3
Estela N B Busanello, Ana C Marques, Noelia Lander, Diogo N de Oliveira, Rodrigo R Catharino, Helena C F Oliveira, Anibal E Vercesi
Statins are efficient cholesterol-lowering medicines utilized worldwide. However, 10% of patients suffer from adverse effects specially related to skeletal muscle function. Pro- or anti-oxidant effects of statins have been reported. Here we hypothesized that statins induce muscle mitochondrial oxidative stress leading to mitochondrial permeability transition (MPT) which may explain statin muscle toxicity. Thus, our aims were to investigate the effects of statin chronic treatment on muscle mitochondrial respiration rates, MPT and redox state indicators in the context of hypercholesterolemia...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28366515/coq10-selective-miscibility-and-penetration-into-lipid-monolayers-with-lower-lateral-packing-density
#4
Sumit Garg, Vandana Swaminathan, Sirisha Dhavala, Michael A Kiebish, Rangaprasad Sarangarajan, Niven R Narain
CoQ10 is ubiquitously present in eukaryotic cells. It acts as electron carrier in the electron transport chain of the inner membrane of the mitochondria to facilitate aerobic cellular respiration. A highly stable lipid nanodispersion formulation containing CoQ10 (BPM31510) is currently in clinical investigation for treatment of cancer. This study was designed to determine whether biophysical interactions between CoQ10 and lipid, in part, explain the observed stability and cellular accumulation of CoQ10 in cells and tissues...
July 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28339599/a-single-nucleotide-polymorphism-in-coq9-affects-mitochondrial-and-ovarian-function-and-fertility-in-holstein-cows%C3%A2
#5
M Sofia Ortega, Stephanie Wohlgemuth, Paula Tribulo, Luiz G B Siqueira, Daniel J Null, John B Cole, Marcus V Da Silva, Peter J Hansen
A single missense mutation at position 159 of coenzyme Q9 (COQ9) (G→A; rs109301586) has been associated with genetic variation in fertility in Holstein cattle, with the A allele associated with higher fertility. COQ9 is involved in the synthesis of coenzyme COQ10, a component of the electron transport system of the mitochondria. Here we tested whether reproductive phenotype is associated with the mutation and evaluated functional consequences for cellular oxygen metabolism, body weight changes, and ovarian function...
March 1, 2017: Biology of Reproduction
https://www.readbyqxmd.com/read/28274308/-lipopolysaccharide-of-porphyromonas-gingivalis-promotes-the-autophagy-of-human-gingival-fibroblasts
#6
Yu Liu, Shujin Li, Senlin Zhang, Xiaoma Cao, Yanmei Zhang
Objective To investigate the impact of lipopolysaccharide of Porphyromonas gingivalis (Pg-LPS) on the autophagy of human gingival fibroblasts (HGFs). Methods Firstly, HGFs was stimulated with 10 μg/mL Pg-LPS for 12 hours or 24 hours. Rapamycin was used as a positive control. The expression of LC3B was detected by Western blotting and the distribution of autophagosomes was observed by indirect immunofluorescence staining. At the same time, mitochondrial ROS (mtROS) was labeled by MitoSOX Red. The levels of mtROS and mitochondrial autophagy were measured in HGFs after treated with Pg-LPS...
March 2017: Xi Bao Yu Fen Zi Mian Yi Xue za Zhi, Chinese Journal of Cellular and Molecular Immunology
https://www.readbyqxmd.com/read/27932481/modeling-monogenic-human-nephrotic-syndrome-in-the-drosophila-garland-cell-nephrocyte
#7
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...
May 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27525823/role-of-mitochondrial-complex-i-and-protective-effect-of-coq10-supplementation-in-propofol-induced-cytotoxicity
#8
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
https://www.readbyqxmd.com/read/27477725/development-of-redox-metabolic-imaging-using-endogenous-molecules
#9
REVIEW
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
https://www.readbyqxmd.com/read/27442316/coenzyme-q10-in-the-treatment-of-corneal-edema-in-kearns-sayre-is-there-an-application-in-fuchs-endothelial-corneal-dystrophy
#10
REVIEW
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
https://www.readbyqxmd.com/read/27155576/disruption-of-the-human-coq5-containing-protein-complex-is-associated-with-diminished-coenzyme-q10-levels-under-two-different-conditions-of-mitochondrial-energy-deficiency
#11
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
https://www.readbyqxmd.com/read/27060254/coenzyme-q-biosynthesis-in-health-and-disease
#12
REVIEW
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
https://www.readbyqxmd.com/read/27015086/characterization-of-a-plasmodium-falciparum-orthologue-of-the-yeast-ubiquinone-binding-protein-coq10p
#13
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
https://www.readbyqxmd.com/read/27012265/coenzyme-q10-and-heart-failure-a-state-of-the-art-review
#14
REVIEW
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
https://www.readbyqxmd.com/read/26946184/accelerated-regeneration-of-atp-level-after-irradiation-in-human-skin-fibroblasts-by-coenzyme-q10
#15
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
https://www.readbyqxmd.com/read/26804019/mitochondrial-dysfunction-in-inherited-renal-disease-and-acute-kidney-injury
#16
REVIEW
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
https://www.readbyqxmd.com/read/26770107/mitochondrial-dysfunction-and-chronic-disease-treatment-with-natural-supplements
#17
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
https://www.readbyqxmd.com/read/26741866/coenzyme-q-10-in-human-health-supporting-evidence
#18
REVIEW
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
https://www.readbyqxmd.com/read/26682233/coenzyme-q10-attenuates-high-glucose-induced-endothelial-progenitor-cell-dysfunction-through-amp-activated-protein-kinase-pathways
#19
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
https://www.readbyqxmd.com/read/26522469/intra-mitochondrial-methylation-deficiency-due-to-mutations-in-slc25a26
#20
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
keyword
keyword
89665
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"