Analysis of coenzyme Q10 in lymphocytes by HPLC–MS/MS

Arias, Ángela; García-Villoria, Judit; Rojo, Ana I.; Bujan, Núria; Briones, Paz; Ribes, Antònia

doi:10.1016/j.jchromb.2012.09.027

Cited by 19 publications

(13 citation statements)

References 16 publications

(22 reference statements)

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“…In this way we demonstrated that it is not necessary to store plasma sample at -20 or -80°C as stated in the literature [1,4,8] for the analysis of CoQ10, thus simplifying the analytical procedure. In fact the analysis of frozen samples is more difficult and longer.…”

Section: Citationmentioning

confidence: 57%

“…It is a benzoquinone with 10 isoprene units in the aliphatic chain [2] and it is essential for electron transfer in the mitochondrial respiratory chain, playing a key role in energy metabolism as an integral part of the electron transport system [3,4]. CoQ10 is the predominant ubiquinone species in humans and it originates from endogenous biosynthesis as well as from dietary intake.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Determination by UPLC/MS-MS of Coenzyme Q10 (CoQ10) in Plasma of Healthy Volunteers before and after Oral Intake of Food Supplements Containing CoQ10

2015

J Anal Bioanal Tech

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Section: Citationmentioning

confidence: 57%

Section: Introductionmentioning

confidence: 99%

Determination by UPLC/MS-MS of Coenzyme Q10 (CoQ10) in Plasma of Healthy Volunteers before and after Oral Intake of Food Supplements Containing CoQ10

2015

J Anal Bioanal Tech

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“…In previously reported LC-MS-based assays for CoQ10 in biological samples, either CoQ11 (14 ), CoQ9 (15)(16)(17)19 ), or dipropoxy-CoQ10 (18 ) has been used as the IS. A potential source of error for MS assays is ionization suppression that varies during a chromatographic run and between the samples.…”

Section: Discussionmentioning

confidence: 99%

Mitochondrial Coenzyme Q10 Determination by Isotope-Dilution Liquid Chromatography–Tandem Mass Spectrometry

2013

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BACKGROUND Coenzyme Q10 (CoQ10) is an essential part of the mitochondrial respiratory chain. Unlike most other respiratory chain disorders, CoQ10 deficiency is potentially treatable. We aimed to develop and validate an accurate liquid chromatography–tandem mass spectrometry (LC-MS/MS) method for the determination of mitochondrial CoQ10 in clinical samples. METHODS We used mitochondria isolated from muscle biopsies of patients (n = 166) suspected to have oxidative phosphorylation deficiency. We also used fibroblast mitochondria from 1 patient with CoQ10 deficiency and 3 healthy individuals. Samples were spiked with nonphysiologic CoQ10-[2H6] internal standard, extracted with 1-propanol and with ethanol and hexane (2 mL/5 mL), and CoQ10 quantified by LC-MS/MS. The method and sample stability were validated. A reference interval was established from the patient data. RESULTS The method had a limit of quantification of 0.5 nmol/L. The assay range was 0.5–1000 nmol/L and the CVs were 7.5%–8.2%. CoQ10 was stable in concentrated mitochondrial suspensions. In isolated mitochondria, the mean ratio of CoQ10 to citrate synthase (CS) activity (CoQ10/CS) was 1.7 nmol/U (95% CI, 1.6–1.7 nmol/U). We suggest a CoQ10/CS reference interval of 1.1–2.8 nmol/U for both sexes and all ages. The CoQ10/CS ratio was 5-fold decreased in fibroblast mitochondria from a patient with known CoQ10 deficiency due to recessive prenyl (decaprenyl) diphosphate synthase, subunit 2 (PDSS2) mutations. CONCLUSIONS Normalization of mitochondrial CoQ10 concentration against citrate synthase activity is likely to reflect most accurately the CoQ10 content available for the respiratory chain. Our assay and the established reference range should facilitate the diagnosis of respiratory chain disorders and treatment of patients with CoQ10 deficiency.

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“…After incubation, cells were trypsinized and washed twice with isotonic buffer. Pelleted cells were resuspended with 300 μL of a buffer solution containing 0.25 mmol/L sucrose, 2 mmol/L EDTA, 10 mmol/L Tris and 100 UI/mL heparin, pH 7.4, and sonicated twice for 5 s. These homogenates were used to determine CoQ 10 biosynthesis measuring by HPLC-MS/MS, as described in Arias et al (2012) [62]. Briefly, HPLC separation was as indicated above and extracted peaks were analyzed by MS/MS in a Micromass Quattro micro™ (Waters/Micromass, Manchester, UK).…”

Section: Analysis Of Coq10 Biosynthesismentioning

confidence: 99%

Biochemical Assessment of Coenzyme Q10 Deficiency

Rodríguez-Aguilera

Cortés

Navas

2017

JCM

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Coenzyme Q10 (CoQ10) deficiency syndrome includes clinically heterogeneous mitochondrial diseases that show a variety of severe and debilitating symptoms. A multiprotein complex encoded by nuclear genes carries out CoQ10 biosynthesis. Mutations in any of these genes are responsible for the primary CoQ10 deficiency, but there are also different conditions that induce secondary CoQ10 deficiency including mitochondrial DNA (mtDNA) depletion and mutations in genes involved in the fatty acid β-oxidation pathway. The diagnosis of CoQ10 deficiencies is determined by the decrease of its content in skeletal muscle and/or dermal skin fibroblasts. Dietary CoQ10 supplementation is the only available treatment for these deficiencies that require a rapid and distinct diagnosis. Here we review methods for determining CoQ10 content by HPLC separation and identification using alternative approaches including electrochemical detection and mass spectrometry. Also, we review procedures to determine the CoQ10 biosynthesis rate using labeled precursors.

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Analysis of coenzyme Q10 in lymphocytes by HPLC–MS/MS

Cited by 19 publications

References 16 publications

Determination by UPLC/MS-MS of Coenzyme Q10 (CoQ10) in Plasma of Healthy Volunteers before and after Oral Intake of Food Supplements Containing CoQ10

Determination by UPLC/MS-MS of Coenzyme Q10 (CoQ10) in Plasma of Healthy Volunteers before and after Oral Intake of Food Supplements Containing CoQ10

Mitochondrial Coenzyme Q10 Determination by Isotope-Dilution Liquid Chromatography–Tandem Mass Spectrometry

Biochemical Assessment of Coenzyme Q10 Deficiency

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