1. Yeast Coq9 controls deamination of coenzyme Q intermediates that derive from para-aminobenzoic acid.

    Biochimica et Biophysica Acta 1851(9):1227 (2015) PMID 26008578 PMCID PMC4516658

    Coq9 is a polypeptide subunit in a mitochondrial multi-subunit complex, termed the CoQ-synthome, required for biosynthesis of coenzyme Q (ubiquinone or Q). Deletion of COQ9 results in dissociation of the CoQ-synthome, but over-expression of Coq8 putative kinase stabilizes the CoQ-synthome in the...
  2. Biochemistry: Unexpected role for vitamin B2.

    Nature 522(7557):427 (2015) PMID 26083748

  3. Isotope-reinforced polyunsaturated fatty acids protect mitochondria from oxidative stress.

    Free Radical Biology and Medicine 82:63 (2015) PMID 25578654

    Polyunsaturated fatty acid (PUFA) peroxidation is initiated by hydrogen atom abstraction at bis-allylic sites and sets in motion a chain reaction that generates multiple toxic products associated with numerous disorders. Replacement of bis-allylic hydrogens of PUFAs with deuterium atoms (D-PUFAs...
  4. Isotope-reinforced polyunsaturated fatty acids protect mitochondria from oxidative stress

    Free Radical Biology and Medicine 82:63 (2015)

    Polyunsaturated fatty acid (PUFA) peroxidation is initiated by hydrogen atom abstraction at bis-allylic sites and sets in motion a chain reaction that generates multiple toxic products associated with numerous disorders. Replacement of bis-allylic hydrogens of PUFAs with deuterium atom...
  5. Resveratrol and para-coumarate serve as ring precursors for coenzyme Q biosynthesis.

    Journal of Lipid Research 56(4):909 (2015) PMID 25681964 PMCID PMC4373747

    Coenzyme Q (Q or ubiquinone) is a redox-active polyisoprenylated benzoquinone lipid essential for electron and proton transport in the mitochondrial respiratory chain. The aromatic ring 4-hydroxybenzoic acid (4HB) is commonly depicted as the sole aromatic ring precursor in Q biosynthesis despite...
  6. Identification of Coq11, a new coenzyme Q biosynthetic protein in the CoQ-synthome in Saccharomyces cerevisiae.

    Journal of Biological Chemistry 290(12):7517 (2015) PMID 25631044 PMCID PMC4367260

    Coenzyme Q (Q or ubiquinone) is a redox active lipid composed of a fully substituted benzoquinone ring and a polyisoprenoid tail and is required for mitochondrial electron transport. In the yeast Saccharomyces cerevisiae, Q is synthesized by the products of 11 known genes, COQ1-COQ9, YAH1, and A...
  7. Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis.

    Biochimica et Biophysica Acta 1841(11):1628 (2014) PMID 25152161 PMCID PMC4331671

    Coq5 catalyzes the only C-methylation involved in the biosynthesis of coenzyme Q (Q or ubiquinone) in humans and yeast Saccharomyces cerevisiae. As one of eleven polypeptides required for Q production in yeast, Coq5 has also been shown to assemble with the multi-subunit complex termed the CoQ-sy...
  8. Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis.

    Biochimica et Biophysica Acta 1841(11):1628 (2014) PMID 25152161 PMCID PMC4331671

    Coq5 catalyzes the only C-methylation involved in the biosynthesis of coenzyme Q (Q or ubiquinone) in humans and yeast Saccharomyces cerevisiae. As one of eleven polypeptides required for Q production in yeast, Coq5 has also been shown to assemble with the multi-subunit complex termed the CoQ-sy...
  9. The metabolite α-ketoglutarate extends lifespan by inhibiting ATP synthase and TOR.

    Nature 510(7505):397 (2014) PMID 24828042 PMCID PMC4263271

    Metabolism and ageing are intimately linked. Compared with ad libitum feeding, dietary restriction consistently extends lifespan and delays age-related diseases in evolutionarily diverse organisms. Similar conditions of nutrient limitation and genetic or pharmacological perturbations of nutrient...
  10. The metabolite α-ketoglutarate extends lifespan by inhibiting ATP synthase and TOR.

    Nature 510(7505):397 (2014) PMID 24828042 PMCID PMC4263271

    Metabolism and ageing are intimately linked. Compared with ad libitum feeding, dietary restriction consistently extends lifespan and delays age-related diseases in evolutionarily diverse organisms. Similar conditions of nutrient limitation and genetic or pharmacological perturbations of nutrient...
  11. Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants.

    Biochimica et Biophysica Acta 1841(4):630 (2014) PMID 24406904 PMCID PMC3959571

    Coenzyme Q biosynthesis in yeast requires a multi-subunit Coq polypeptide complex. Deletion of any one of the COQ genes leads to respiratory deficiency and decreased levels of the Coq4, Coq6, Coq7, and Coq9 polypeptides, suggesting that their association in a high molecular mass complex is requi...
  12. Osmotic stress: Is CoQ a membrane stabilizer?

    Nature Chemical Biology 10(4):242 (2014) PMID 24643237

  13. Osmotic stress: Is CoQ a membrane stabilizer?

    Nature Chemical Biology 10(4):242 (2014) PMID 24643237

  14. Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeastcoqnull mutants

    Biochimica et Biophysica Acta (BBA) - Molecular... 1841(4):630 (2014)

    Coenzyme Q biosynthesis in yeast requires a multi-subunit Coq polypeptide complex. Deletion of any one of the COQ genes leads to respiratory deficiency and decreased levels of the Coq4, Coq6, Coq7, and Coq9 polypeptides, suggesting that their association in a high molecular mass comple...
  15. Unusual kinetic isotope effects of deuterium reinforced polyunsaturated fatty acids in tocopherol-mediated free radical chain oxidations.

    Journal of the American Chemical Society 136(3):838 (2014) PMID 24380377

    Substitution of -CD2- at the reactive centers of linoleic and linolenic acids reduces the rate of abstraction of D by a tocopheryl radical by as much as 36-fold, compared to the abstraction of H from a corresponding -CH2- center. This H atom transfer reaction is the rate-determining step in the ...
  16. Unusual kinetic isotope effects of deuterium reinforced polyunsaturated fatty acids in tocopherol-mediated free radical chain oxidations.

    Journal of the American Chemical Society 136(3):838 (2014) PMID 24380377

    Substitution of -CD2- at the reactive centers of linoleic and linolenic acids reduces the rate of abstraction of D by a tocopheryl radical by as much as 36-fold, compared to the abstraction of H from a corresponding -CH2- center. This H atom transfer reaction is the rate-determining step in the ...
  17. List of Contributors
    Serge Alfos, Modhi Ali S. Alshammari, Belmira Lara da Silveira Andrade Costa, Se Min Bang, Matthew R. Battistini, Cheryl Tatano Beck, Juliana Maria Carrazone Borba, J. Thomas Brenna, Nicole Burca, Philip C. Calder, Paolo Capodaglio, Henriqueta Dias Cardoso, Nicola Cau, H.M. Chandola, Caroline E. Childs, Kyoung Sang Cho, Veronica Cimolin, Catherine F. Clarke, Adriana Coppola, Georgina E. Crichton, Lisette C.P.G.M. de Groot, Daniel R. Dempsey, Patricia Coelho de Velasco, Ana Francisca Diallo, Fabiana Di Sabatino, Simon C. Dyall, Merrill F. Elias, Akhlaq A. Farooqui, Emma K. Farrell, Alessandro Ferretti, Heather M. Francis, Linnea R. Freeman, Dina Gazizova, Manuela Galli, Carmine Gazzaruso, Grace E. Giles, Catarina Gonçalves-Pimentel, Amanda L. Goodell, Rubem Carlos Araújo Guedes, Kei Hamazaki, Tomohito Hamazaki, Lucas W. Hernandez, Ted M. Hsu, Mary C. Hunt, Hidekuni Inadera, Kristen A. Jeffries, Michelle Price Judge, Robin B. Kanarek, Scott E. Kanoski, Lauren E. Lawson, Peter Lembke, Caroline R. Mahoney, Amy B. Manning-Boğ, Joshua C. McBride, David J. Merkler, Perry Mitchell, Robert J. Molinari, Kazuo Nakamoto, Etsuo Niki, Maria Chiara Paolino, Christopher Papandreou, Pasquale Parisi, Rita Paroni, Priscila Pereira Passos, Gaurav Paul, Steven Pennell, Anna Petroni, Basant K. Puri, Stanley I. Rapoport, Suresh I.S. Rattan, Patrizia Risé, Gilbert Lujan Rivera, Vitaly A. Roginsky, Eraldo Fonseca Santos, Anett Schubert, Claudio Alberto Serfaty, Mikhail S. Shchepinov, Mototada Shichiri, Vijay Karam Singh, R.J. Stevenson, Ameer Taha, Zaldy S. Tan, Ila Tanna, Randy To, Amanda Toh, Shogo Tokuyama, Hui Tsui, Ondine van de Rest, Alyssa Bianca Velasco, Alberto Verrotti, Luca Vismara, Dominik P. Waluk, Ronald Ross Watson, and Yasukazu Yoshida

  18. Chapter 31 - Deuterium Protection of Polyunsaturated Fatty Acids against Lipid Peroxidation: A Novel Approach to Mitigating Mitochondrial Neurological Diseases

    Oxidative stress is associated with numerous neurological disorders. Mitochondrial malfunction contributes to generation of reactive oxygen species (ROS) in proximity to mitochondrial membranes rich in polyunsaturated fatty acids (PUFAs). ROS initiate PUFA autoxidation, a chain reaction ...
  19. ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.

    Journal of Clinical Investigation 123(12):5179 (2013) PMID 24270420 PMCID PMC3859425

    Identification of single-gene causes of steroid-resistant nephrotic syndrome (SRNS) has furthered the understanding of the pathogenesis of this disease. Here, using a combination of homozygosity mapping and whole human exome resequencing, we identified mutations in the aarF domain containing kin...
  20. ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.

    Journal of Clinical Investigation 123(12):5179 (2013) PMID 24270420 PMCID PMC3859425

    Identification of single-gene causes of steroid-resistant nephrotic syndrome (SRNS) has furthered the understanding of the pathogenesis of this disease. Here, using a combination of homozygosity mapping and whole human exome resequencing, we identified mutations in the aarF domain containing kin...