Biochemistry of Mitochondrial Coenzyme Q Biosynthesis.
Reads0
Chats0
TLDR
This work highlights recent progress toward filling knowledge gaps in CoQ biosynthesis through both traditional biochemistry and cutting-edge 'omics' approaches and presents questions framed by the recently discovered Co Q biosynthesis complex and by putative biophysical barriers.About:
This article is published in Trends in Biochemical Sciences.The article was published on 2017-10-01 and is currently open access. It has received 197 citations till now.read more
Citations
More filters
Journal ArticleDOI
Clinico‐Genetic, Imaging and Molecular Delineation of COQ8A‐Ataxia: A Multicenter Study of 59 Patients
Andreas Traschütz,Andreas Traschütz,Tommaso Schirinzi,Lucia Laugwitz,Lucia Laugwitz,Nathan H. Murray,Nathan H. Murray,Craig A. Bingman,Craig A. Bingman,Selina Reich,Selina Reich,Jan Kern,Anna Heinzmann,Gessica Vasco,Enrico Bertini,Ginevra Zanni,Alexandra Durr,Stefania Magri,Franco Taroni,Alessandro Malandrini,Jonathan Baets,Peter De Jonghe,Willem De Ridder,Matthieu Bereau,Matthieu Bereau,Stephanie Demuth,Christos Ganos,A. Nazli Basak,Hasmet Hanagasi,Semra Hız Kurul,Benjamin Bender,Ludger Schöls,Ludger Schöls,Ute Grasshoff,Thomas Klopstock,Thomas Klopstock,Rita Horvath,Rita Horvath,Bart P.C. van de Warrenburg,Lydie Burglen,Christelle Rougeot,Claire Ewenczyk,Michel Koenig,Filippo M. Santorelli,Mathieu Anheim,Renato P. Munhoz,Tobias B. Haack,Felix Distelmaier,David J. Pagliarini,David J. Pagliarini,Hélène Puccio,Matthis Synofzik,Matthis Synofzik +52 more
TL;DR: The clinicogenetic, molecular, and neuroimaging spectrum of COQ8A‐ataxia in a large worldwide cohort is mapped, and first progression data is provided, including treatment response to coenzyme Q10 (CoQ10).
Cardiofaciocutaneous (CFC) syndrome associated with muscular coenzyme Q(10) deficiency
Alec Aeby,Yves Sznajer,Hélène Cavé,Elisabeth Rebuffat,Rudy Van Coster,O Rigal,P. Van Bogaert +6 more
TL;DR: The case of a 4-year-old girl who presented a CFC syndrome, confirmed by the presence of a pathogenic R257Q BRAF gene mutation, together with a muscular CoQ10 deficiency, highlights that Co Q10 might contribute to the pathogenesis of CFC Syndrome.
Journal ArticleDOI
Biosynthesis and applications of prenylquinones
TL;DR: The synthesis and applications of prenylquinones are reviewed, including for phylloquinone (PhQ), which has a phytyl moiety instead of an isoprenyl tail.
Journal ArticleDOI
A Soluble Metabolon Synthesizes the Isoprenoid Lipid Ubiquinone.
M. Hajj Chehade,Ludovic Pelosi,Cameron David Fyfe,Laurent Loiseau,Bérengère Rascalou,Sabine Brugière,Katayoun Kazemzadeh,Chau-Duy-Tam Vo,Lidia Ciccone,Laurent Aussel,Yohann Couté,Marc Fontecave,Frédéric Barras,Murielle Lombard,Fabien Pierrel +14 more
TL;DR: It is demonstrated that seven Ubi proteins form the Ubi complex, a stable metabolon that catalyzes the last six reactions of the UQ biosynthetic pathway in Escherichia coli and highlights how the supramolecular organization of soluble enzymes allows the modification of hydrophobic substrates in a hydrophilic environment.
Journal ArticleDOI
Multi-omic Mitoprotease Profiling Defines a Role for Oct1p in Coenzyme Q Production
Mike T. Veling,Mike T. Veling,Andrew G. Reidenbach,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,David J. Pagliarini +14 more
TL;DR: The Oct1p processing of Coq5p as an essential post-translational event for proper CoQ production is defined and the data visualization tool enables efficient exploration of mitoprotease profiles that can serve as the basis for future mechanistic investigations.
References
More filters
Journal ArticleDOI
A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology
David J. Pagliarini,Sarah E. Calvo,Betty Chang,Sunil A Sheth,Scott Bradley Vafai,Shao En Ong,Geoffrey A. Walford,Canny Sugiana,Avihu Boneh,Avihu Boneh,William K. Chen,David E. Hill,Marc Vidal,James G. Evans,David R. Thorburn,David R. Thorburn,Steven A. Carr,Vamsi K. Mootha,Vamsi K. Mootha +18 more
TL;DR: This work predicts 19 proteins to be important for the function of complex I (CI) of the electron transport chain and validate a subset of these predictions using RNAi, including C8orf38, which is shown to have an inherited mutation in a lethal, infantile CI deficiency.
Journal ArticleDOI
Proteomic Mapping of Mitochondria in Living Cells via Spatially Restricted Enzymatic Tagging
Hyun-Woo Rhee,Peng Zou,Namrata D. Udeshi,Jeffrey D. Martell,Vamsi K. Mootha,Vamsi K. Mootha,Steven A. Carr,Alice Y. Ting,Alice Y. Ting +8 more
TL;DR: An approach that bridges microscopy and proteomics to produce a spatially and temporally resolved proteomic map of mitochondria from living cells using a genetically targetable peroxidase enzyme that biotinylates nearby proteins, which are subsequently purified and identified by MS.
Proteomic Mapping of Mitochondria in Living Cells via Spatially Restricted Enzymatic Tagging
Hyun-Woo Rhee,Peng Zou,Namrata D. Udeshi,Jeffrey D. Martell,Vamsi K. Mootha,Vamsi K. Mootha,Steven A. Carr,Alice Y. Ting,Alice Y. Ting +8 more
TL;DR: In this article, a peroxidase-based method was used to identify 495 proteins within the human mitochondrial matrix, including 31 proteins not previously linked to mitochondria, and the labeling was exceptionally specific and distinguished between inner membrane proteins facing the matrix versus the intermembrane space.
Journal ArticleDOI
Ubiquinol-10 protects human low density lipoprotein more efficiently against lipid peroxidation than does alpha-tocopherol
TL;DR: Investigation of the temporal disappearance of natural antioxidants associated with human low density lipoprotein (LDL) in relation to the appearance of various classes of lipid hydroperoxides shows that ubiquinol-10 is much more efficient in inhibiting LDL oxidation than either lycopene, beta-carotene, or alpha-tocopherol.
Journal ArticleDOI
Mitochondrial disorders as windows into an ancient organelle
TL;DR: These disorders are reviewed and explored in the context of a contemporary understanding of mitochondrial evolution, biochemistry and genetics to inspire the development of drug treatments for rare and common diseases.
Related Papers (5)
Mitochondrial COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis
Danielle C. Lohman,Farhad Forouhar,Emily T. Beebe,Matthew S. Stefely,Catherine E. Minogue,Arne Ulbrich,Jonathan A. Stefely,Shravan Sukumar,Marta Luna-Sánchez,Adam Jochem,Scott Lew,Jayaraman Seetharaman,Rong Xiao,Huang Wang,Huang Wang,Michael S. Westphall,Russell L. Wrobel,John Everett,John Everett,Julie C. Mitchell,Luis C. López,Joshua J. Coon,Liang Tong,David J. Pagliarini +23 more
ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption
Shazia Ashraf,Heon Yung Gee,Stéphanie Woerner,Stéphanie Woerner,Letian X. Xie,Virginia Vega-Warner,Svjetlana Lovric,Humphrey Fang,Xuewen Song,Daniel C. Cattran,Carmen Avila-Casado,Andrew D. Paterson,Patrick Nitschké,Christine Bole-Feysot,Pierre Cochat,Julian Esteve-Rudd,Birgit Haberberger,Susan J. Allen,Weibin Zhou,Rannar Airik,Edgar A. Otto,Moumita Barua,Mohamed H Al-Hamed,Jameela A. Kari,Jonathan Evans,Agnieszka Bierzynska,Moin A. Saleem,Detlef Bockenhauer,Robert Kleta,Sherif El Desoky,Duygu Övünç Hacıhamdioğlu,Faysal Gok,Joseph Washburn,Roger C. Wiggins,Murim Choi,Murim Choi,Richard P. Lifton,Richard P. Lifton,Shawn Levy,Zhe Han,Leonardo Salviati,Holger Prokisch,David S. Williams,Martin R. Pollak,Catherine F. Clarke,York Pei,Corinne Antignac,Corinne Antignac,Friedhelm Hildebrandt,Friedhelm Hildebrandt,Friedhelm Hildebrandt +50 more
COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness
Saskia F. Heeringa,Gil Chernin,Moumita Chaki,Weibin Zhou,Alexis Sloan,Ziming Ji,Letian X. Xie,Leonardo Salviati,Toby W. Hurd,Virginia Vega-Warner,Paul D. Killen,Yehoash Raphael,Shazia Ashraf,Bugsu Ovunc,Dominik S. Schoeb,Heather M. McLaughlin,Rannar Airik,Christopher N. Vlangos,Rasheed Gbadegesin,Bernward Hinkes,Pawaree Saisawat,Eva Trevisson,Mara Doimo,Alberto Casarin,Vanessa Pertegato,Gianpietro Giorgi,Holger Prokisch,Agnès Rötig,Gudrun Nürnberg,Christian Becker,Su Q. Wang,Fatih Ozaltin,Rezan Topaloglu,Aysin Bakkaloglu,Sevcan A. Bakkaloglu,Dominik N. Müller,Antje Beissert,Sevgi Mir,Afig Berdeli,Seza ϖzen,Martin Zenker,Verena Matejas,Carlos Santos-Ocaña,Plácido Navas,Takehiro Kusakabe,Andreas Kispert,Sema Akman,Neveen A. Soliman,Stefanie Krick,Peter Mundel,Jochen Reiser,Peter Nürnberg,Catherine F. Clarke,Roger C. Wiggins,Christian Faul,Friedhelm Hildebrandt +55 more