Literature DB >> 34118048

Mitochondrial Coenzyme Q10 Determination Via Isotope Dilution Liquid Chromatography -Tandem Mass Spectrometry.

Erik Hedman1, Outi Itkonen2.   

Abstract

Coenzyme Q10 (CoQ10) is an essential part of the mitochondrial respiratory chain . Here, we describe an accurate and sensitive liquid chromatography tandem mass spectrometry (LC-MS/MS) method for determination of mitochondrial CoQ10 in isolated mitochondria . In the assay, mitochondrial suspensions are spiked with CoQ10-[2H9] internal standard (IS), extracted with organic solvents and CoQ10 quantified by LC-MS/MS using multiple reaction monitoring (MRM).

Entities:  

Keywords:  Coenzyme Q10; Isotope dilution; LC-MS/MS; Mitochondrial disease; Ubiquinone

Mesh：

Substances：

Year:  2021        PMID： 34118048     DOI： 10.1007/978-1-0716-1262-0_21

Source DB:  PubMed          Journal:  Methods Mol Biol        ISSN： 1064-3745


  28 in total

1.  Coenzyme q10 prevents apoptosis by inhibiting mitochondrial depolarization independently of its free radical scavenging property.

Authors:  Laura Papucci; Nicola Schiavone; Ewa Witort; Martino Donnini; Andrea Lapucci; Alessio Tempestini; Lucia Formigli; Sandra Zecchi-Orlandini; Giovanni Orlandini; Giuseppe Carella; Rosario Brancato; Sergio Capaccioli
Journal:  J Biol Chem       Date:  2003-05-07       Impact factor: 5.157

2.  The kinetics of the redox reactions of ubiquinone related to the electron-transport activity in the respiratory chain.

Authors:  A Kröger; M Klingenberg
Journal:  Eur J Biochem       Date:  1973-04

3.  Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis.

Authors:  José M López-Martín; Leonardo Salviati; Eva Trevisson; Giovanni Montini; Salvatore DiMauro; Catarina Quinzii; Michio Hirano; Angeles Rodriguez-Hernandez; Mario D Cordero; José A Sánchez-Alcázar; Carlos Santos-Ocaña; Plácido Navas
Journal:  Hum Mol Genet       Date:  2007-03-20       Impact factor: 6.150

4.  COQ4 Mutation Leads to Childhood-Onset Ataxia Improved by CoQ10 Administration.

Authors:  Ahmet Okay Caglayan; Hakan Gumus; Erin Sandford; Thomas L Kubisiak; Qianyi Ma; A Bilge Ozel; Huseyin Per; Jun Z Li; Vikram G Shakkottai; Margit Burmeister
Journal:  Cerebellum       Date:  2019-06       Impact factor: 3.847

Review 5.  Heterogeneity of coenzyme Q10 deficiency: patient study and literature review.

Authors:  Valentina Emmanuele; Luis C López; Luis López; Andres Berardo; Ali Naini; Saba Tadesse; Bing Wen; Erin D'Agostino; Martha Solomon; Salvatore DiMauro; Catarina Quinzii; Michio Hirano
Journal:  Arch Neurol       Date:  2012-08

6.  Requirement for coenzyme Q in plasma membrane electron transport.

Authors:  I L Sun; E E Sun; F L Crane; D J Morré; A Lindgren; H Löw
Journal:  Proc Natl Acad Sci U S A       Date:  1992-12-01       Impact factor: 11.205

Review 7.  Ubiquinol: an endogenous antioxidant in aerobic organisms.

Authors:  L Ernster; P Forsmark-Andrée
Journal:  Clin Investig       Date:  1993

8.  Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4-dihydroxybensoic acid.

Authors:  Christoph Freyer; Henrik Stranneheim; Karin Naess; Arnaud Mourier; Andrea Felser; Camilla Maffezzini; Nicole Lesko; Helene Bruhn; Martin Engvall; Rolf Wibom; Michela Barbaro; Yvonne Hinze; Måns Magnusson; Robin Andeer; Rolf H Zetterström; Ulrika von Döbeln; Anna Wredenberg; Anna Wedell
Journal:  J Med Genet       Date:  2015-06-17       Impact factor: 6.318

9.  A fatal case of COQ7-associated primary coenzyme Q10 deficiency.

Authors:  Anna K-Y Kwong; Annie T-G Chiu; Mandy H-Y Tsang; Kin-Shing Lun; Richard J T Rodenburg; Jan Smeitink; Brian H-Y Chung; Cheuk-Wing Fung
Journal:  JIMD Rep       Date:  2019-04-03

10.  Intra-mitochondrial Methylation Deficiency Due to Mutations in SLC25A26.

Authors:  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
Journal:  Am J Hum Genet       Date:  2015-10-29       Impact factor: 11.025
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