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1. Full Text Coenzyme Q biosynthesis in health and disease
by Acosta, Manuel Jesús and Vazquez Fonseca, Luis and Desbats, Maria Andrea and Cerqua, Cristina and Zordan, Roberta and Trevisson, Eva and Salviati, Leonardo
Biochimica et biophysica acta. Bioenergetics, ISSN 0005-2728, 08/2016, Volume 1857, Issue 8, pp. 1079 - 1085
Mitochondrial disorders | Coenzyme Q | Ubiquinone | Coenzyme Q10 deficiency | Steroid resistant nephrotic syndrome | Adenosine Triphosphate - deficiency | Electron Transport | Reactive Oxygen Species - metabolism | Saccharomyces cerevisiae - genetics | Humans | Protein Multimerization | Ubiquinone - deficiency | Mitochondrial Diseases - metabolism | Ubiquinone - metabolism | Adenosine Triphosphate - agonists | Adenosine Triphosphate - biosynthesis | Muscle Weakness - genetics | Ubiquinone - biosynthesis | Saccharomyces cerevisiae - metabolism | Mitochondria - genetics | Ataxia - drug therapy | Ubiquinone - therapeutic use | Ataxia - genetics | Ataxia - metabolism | Mitochondrial Diseases - genetics | Ataxia - physiopathology | Mitochondrial Diseases - drug therapy | Electron Transport Chain Complex Proteins - genetics | Mitochondria - metabolism | Ubiquinone - genetics | Reactive Oxygen Species - antagonists & inhibitors | Animals | Electron Transport Chain Complex Proteins - metabolism | Muscle Weakness - drug therapy | Muscle Weakness - metabolism | Muscle Weakness - physiopathology | Mutation | Mitochondrial Diseases - physiopathology | Physiological aspects | Enzymes | Phosphatases | Cytochrome b | Permeability | Quinone | Antioxidants | Genes | Kidney diseases
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2. Full Text Cryo-em structures of complex i from mouse heart mitochondria in two biochemically defined states
by Agip, Ahmed-Noor A and Blaza, James N and Bridges, Hannah R and Viscomi, Carlo and Rawson, Shaun and Muench, Stephen P and Hirst, Judy
Nature structural & molecular biology, ISSN 1545-9993, 06/2018, Volume 25, Issue 7, pp. 548 - 556
Protein Subunits | Mitochondria, Heart - enzymology | Models, Molecular | Phospholipids - chemistry | Ubiquinone - metabolism | NADH Dehydrogenase - ultrastructure | NADH Dehydrogenase - chemistry | NADH Dehydrogenase - metabolism | Nucleotides - metabolism | Electron Transport Complex I - metabolism | Nucleotides - chemistry | Cryoelectron Microscopy | Protein Structural Elements | Electron Transport Complex I - chemistry | Animals | Protein Structure, Quaternary | Electron Transport Complex I - ultrastructure | Mice | Enzyme Activation | Phospholipids - metabolism | Binding Sites | Ubiquinone - chemistry | Heart | Mitochondria | Usage | NADH dehydrogenase | Physiological aspects | Models | Electron microscopy | Protons | NADH-ubiquinone oxidoreductase | Phosphorylation | Animal models | Oxidoreductase | Homology | Mammals | Electron transport chain | Ubiquinone | Reperfusion | NADH | Ischemia | Oxidative phosphorylation | Rodents | Nicotinamide adenine dinucleotide | Ubiquinone oxidoreductase
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3. Full Text ADCK4 mutations promote steroid-Resistant nephrotic syndrome through CoQ10 biosynthesis disruption
by Ashraf, Shazia and Gee, Heon Yung and Woerner, Stephanie and Xie, Letian X and Vega-Warner, Virginia and Lovric, Svjetlana and Fang, Humphrey and Song, Xuewen and Cattran, Daniel C and Avila-Casado, Carmen and Paterson, Andrew D and Nitschké, Patrick and Bole-Feysot, Christine and Cochat, Pierre and Esteve-Rudd, Julian and Haberberger, Birgit and Allen, Susan J and Zhou, Weibin and Airik, Rannar and Otto, Edgar A and Barua, Moumita and Al-Hamed, Mohamed H and Kari, Jameela A and Evans, Jonathan and Bierzynska, Agnieszka and Saleem, Moin A and Böckenhauer, Detlef and Kleta, Robert and Desoky, Sherif El and Hacihamdioglu, Duygu O and Gok, Faysal and Washburn, Joseph and Wiggins, Roger C and Choi, Murim and Lifton, Richard P and Levy, Shawn and Han, Zhe and Salviati, Leonardo and Prokisch, Holger and Williams, David S and Pollak, Martin and Clarke, Catherine F and Pei, York and Antignac, Corinne and Hildebrandt, Friedhelm
The Journal of clinical investigation, ISSN 0021-9738, 12/2013, Volume 123, Issue 12, pp. 5179 - 5189
Protein Kinases - genetics | Humans | Nephrotic Syndrome - metabolism | Molecular Sequence Data | Ubiquinone - metabolism | Nephrotic Syndrome - genetics | Nephrotic Syndrome - drug therapy | Adrenal Cortex Hormones - therapeutic use | Zebrafish - embryology | Gene Knockdown Techniques | Ubiquinone - biosynthesis | Young Adult | DNA Mutational Analysis | Conserved Sequence | Ubiquinone - therapeutic use | Protein Kinases - deficiency | Child | Drosophila Proteins - antagonists & inhibitors | Drug Resistance | Disease Models, Animal | Fibroblasts - metabolism | Amino Acid Sequence | Podocytes - metabolism | Ubiquinone - analogs & derivatives | Zebrafish Proteins - antagonists & inhibitors | Cells, Cultured | Adrenal Cortex Hormones - pharmacology | Nephrotic Syndrome - pathology | Rats | Ubiquinone - antagonists & inhibitors | Zebrafish - genetics | Sequence Homology, Amino Acid | Exome - genetics | Podocytes - ultrastructure | Sequence Alignment | Animals | Protein Kinases - physiology | Adolescent | Consanguinity | Drosophila Proteins - genetics | Mutation | Zebrafish Proteins - genetics | Mitochondria - physiology | Biosynthesis | Nephrotic syndrome | Genetic aspects | Research | Gene mutations | Index Medicus | Abridged Index Medicus
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4. Full Text Clinical syndromes associated with Coenzyme Q 10 deficiency
by Alcázar-Fabra, María and Trevisson, Eva and Brea-Calvo, Gloria
Essays in biochemistry, ISSN 0071-1365, 07/2018, Volume 62, Issue 3, pp. 377 - 398
Mitochondrial Diseases - genetics | Ataxia - physiopathology | Ataxia - therapy | Muscle Weakness - diagnosis | Humans | Ubiquinone - analogs & derivatives | Ubiquinone - deficiency | Genotype | Structure-Activity Relationship | Ubiquinone - genetics | Ataxia - diagnosis | Muscle Weakness - genetics | Syndrome | Ubiquinone - biosynthesis | Phenotype | Ubiquinone - physiology | Mitochondrial Diseases - therapy | Muscle Weakness - physiopathology | Muscle Weakness - therapy | Mutation | Ataxia - genetics | Mitochondrial Diseases - physiopathology | Mitochondrial Diseases - diagnosis | Ubiquinone - chemistry
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5. Full Text Bioavailability of coenzyme Q10 supplements depends on carrier lipids and solubilization
by López-Lluch, Guillermo and del Pozo-Cruz, Jesús and Sánchez-Cuesta, Ana and Cortés-Rodríguez, Ana Belén and Navas, Plácido
Nutrition (Burbank, Los Angeles County, Calif.), ISSN 0899-9007, 01/2019, Volume 57, pp. 133 - 140
Human | Ubiquinol | Ubiquinone | Bioavailability | Coenzyme Q10 | Life Sciences & Biomedicine | Nutrition & Dietetics | Science & Technology | Ubiquinone - administration & dosage | Ubiquinone - pharmacokinetics | Double-Blind Method | Administration, Oral | Area Under Curve | Humans | Ubiquinone - analogs & derivatives | Solubility | Biological Availability | Lipids | Male | Drug Carriers | Cross-Over Studies | Young Adult | Capsules | Adolescent | Adult | Female | Dietary Supplements | Ubiquinone - chemistry | Antioxidants | Enzymes | Plasma | Preservatives | Physiological effects | Ascorbic acid | Statistical analysis | Dehydrogenases | Solubilization | Chromatography | Ingestion | Lipoproteins | Physiological factors | Physiology | Drug dosages | Crystal structure
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6. Full Text COQ11 deletion mitigates respiratory deficiency caused by mutations in the gene encoding the coenzyme Q chaperone protein Coq10
by Bradley, Michelle C and Yang, Krista and Fernandez-del-Rio, Lucia and Ngo, Jennifer and Ayer, Anita and Tsui, Hui S and Novales, Noelle Alexa and Stocker, Roland and Shirihai, Orian S and Barros, Mario H and Clarke, Catherine F
The Journal of biological chemistry, ISSN 0021-9258, 05/2020, Volume 295, Issue 18, pp. 6023 - 6042
Life Sciences & Biomedicine | Biochemistry & Molecular Biology | Science & Technology | Gene Expression Regulation, Fungal | Saccharomyces cerevisiae - genetics | Humans | Ubiquinone - analogs & derivatives | Ubiquinone - deficiency | Ubiquinone - metabolism | Mitochondria - metabolism | Ubiquinone - genetics | Saccharomyces cerevisiae Proteins - genetics | Gene Knockout Techniques | Protein Transport | Ubiquinone - biosynthesis | Saccharomyces cerevisiae - cytology | Saccharomyces cerevisiae - metabolism | Gene Deletion | Saccharomyces cerevisiae Proteins - metabolism | Index Medicus
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7. Full Text Coenzyme Q10 as a therapy for mitochondrial disease
by Hargreaves, I.P
The international journal of biochemistry & cell biology, ISSN 1357-2725, 04/2014, Volume 49, Issue 1, pp. 105 - 111
Oxidative stress | Idebenone | EPI-743 | Coenzyme Q10 | Mitochondrial respiratory chain | Mitochondrial Diseases - drug therapy | Animals | Humans | Muscle Weakness - drug therapy | Ubiquinone - analogs & derivatives | Ubiquinone - deficiency | Ataxia - drug therapy | Ubiquinone - therapeutic use | Treatment Outcome | Molecular Structure | Ubiquinone - chemistry | Index Medicus
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8. Full Text Molecular genetics of ubiquinone biosynthesis in animals
by Wang, Ying and Hekimi, Siegfried
Critical reviews in biochemistry and molecular biology, ISSN 1040-9238, 2/2013, Volume 48, Issue 1, pp. 69 - 88
Ubiquinone biosynthesis | metazoan model systems | COQ genes | ubiquinone deficiency | unicellular model systems | human patients | coenzyme Q | Human patients | Unicellular model systems | Coenzyme Q | Metazoan model systems | Ubiquinone deficiency | Life Sciences & Biomedicine | Biochemistry & Molecular Biology | Science & Technology | Mitochondrial Diseases - genetics | Humans | Ubiquinone - deficiency | Mitochondrial Diseases - metabolism | Ubiquinone - metabolism | Ubiquinone - genetics | Muscle Weakness - genetics | Phenotype | Animals | Muscle Weakness - metabolism | Mutation | Ataxia - genetics | Ataxia - metabolism | Ubiquinone - chemistry | Index Medicus | ubiquinone biosynthesis
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9. Full Text Locking loop movement in the ubiquinone pocket of complex I disengages the proton pumps
by Cabrera Orefice, A and Yoga, Etienne Galemou and Wirth, C and Siegmund, K and Zwicker, K and Guerrero Castillo, S and Hunte, C and Brandt, U
Nature communications, ISSN 2041-1723, 2018, Volume 9, Issue 1, pp. 4500 - 10
Science & Technology - Other Topics | Multidisciplinary Sciences | Science & Technology | Electron Transport | Mutagenesis, Site-Directed | Reactive Oxygen Species - metabolism | Yarrowia - metabolism | Proton Pumps - chemistry | Disulfides | Models, Molecular | Crystallography, X-Ray | Proton Pumps - ultrastructure | Molecular Dynamics Simulation | Mitochondrial Membranes - metabolism | Electron Transport Complex I - chemistry | Electron Transport Complex I - genetics | Escherichia coli - genetics | Escherichia coli - metabolism | Escherichia coli Proteins - genetics | Electron Transport Complex I - ultrastructure | Protein Conformation | Mitochondrial Membranes - enzymology | Enzyme Activation | Kinetics | Ubiquinone - ultrastructure | Ubiquinone - chemistry | Yarrowia - genetics | Protons | Binding | NADH-ubiquinone oxidoreductase | Reactive oxygen species | Disulfide bonds | Oxidoreductase | Electron transfer | Proteins | Electron transport chain | Mitochondria | Ubiquinone | NADH | Mutagenesis | Pumping | Site-directed mutagenesis | Energy conversion | Nicotinamide adenine dinucleotide | Ubiquinone oxidoreductase | Index Medicus
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10. Full Text Understanding Ubiquinone
by Wang, Ying and Hekimi, Siegfried
Trends in cell biology, ISSN 0962-8924, 2016, Volume 26, Issue 5, pp. 367 - 378
Pathology | ubiquinone | electron transport | mitochondria | reactive oxygen species | coenzyme Q | Coenzyme Q | Mitochondria | Reactive oxygen species | Ubiquinone | Electron transport | Life Sciences & Biomedicine | Science & Technology | Cell Biology | Animals | Reactive Oxygen Species - metabolism | Energy Metabolism | Signal Transduction | Antioxidants - metabolism | Humans | Ubiquinone - metabolism | Mitochondria - metabolism | Ubiquinone - chemistry | Ubiquinone - biosynthesis | Enzymes | Genetic engineering | Index Medicus
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