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Coenzyme Q10 (CoQ10) is an important cofactor in the mitochondrial respiratory chain and a potent endogenous antioxidant. CoQ10 deficiency is currently associated with numerous diseases like mitochondrial and neurodegenerative pathologies, in which the earliest diagnosis and treatment with CoQ10 supplementation becomes paramount for patient's treatment. Consequently, the determination of CoQ10 levels in different biological matrices positions as a fundamental tool. Urine is an attractive and non-invasive alternative source to tissue, blood or other biofluids for CoQ10 analysis. However, it poses an analytical challenge, as it generally requires a complex sample preparation, with multiple steps. In this work we developed and validated a molecularly imprinted polymer solid phase extraction (MIP-SPE) followed by a HPLC-MS/MS method for the analysis of CoQ10 in urine. The MIP-SPE method developed is simple and fast compared to previously traditional reported methods, with reduced processing time, improved sample cleaning and excellent recovery values, along with its inherent high selectivity. The developed chromatographic method was validated according to FDA guidelines, and demonstrated to be suitable for the analysis of CoQ10 in urine samples with LOQ and LOD values of 0.6 ng/mL and 0.2 ng/mL of CoQ10 in urine respectively. Recovery values at three concentration levels were higher than 90.0%.The proposed method is amenable to be applied in pediatric patients due to the low sample requirement and useful for diagnosis and post-treatment control.
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http://dx.doi.org/10.1016/j.jchromb.2019.03.029 | DOI Listing |
Brain Dev
August 2025
Department of Pediatrics, Kyorin University School of Medicine, Tokyo, Japan.
Background: In some countries, glutaric acidemia type 1 (GA1) is included in newborn screening (NBS) panels using tandem mass spectrometry (MS/MS) analysis of acylcarnitine. However, the low excretor phenotype of glutaric acidemia type 1 (LE-GA1) has been increasingly recognaized and may lead to false-negative NBS results and can be missed by urine organic acid and plasma acylcarnitine profile analyses.
Case: We report a case of LE-GA1 with an atypical imaging course.
Int J Mol Sci
May 2025
Research Centre for Medical Genetics, 115522 Moscow, Russia.
The m.13513G>A (p.Asp393Asn) substitution in the (Mitochondrially Encoded NADH/Ubiquinone Oxidoreductase Core Subunit 5) gene is a common pathogenic variant associated with primary mitochondrial disorders.
View Article and Find Full Text PDFAnim Microbiome
April 2025
Key Laboratory of Agro-Ecological Processes in Subtropical Region, National Engineering Laboratory for Pollution Control and Waste Utilization in Livestock and Poultry Production, Hunan Provincial Key Laboratory of Animal Nutritional Physiology and Metabolic Process, Institute of Subtropical Agricul
Background: Cooling is one of the most common and economical methods to ameliorate heat stress (HS), and it has been discovered to alter the lipopolysaccharide (LPS) endotoxin level in ruminants. However, whether the endotoxin variation induced by cooling relates to the quorum sensing (QS) within the ruminal microflora remains unknown. The current study was consequently performed to examine whether cooling could influence the endotoxin distribution across different biofluids, ruminal microbiota, and ruminal metabolisms through affecting the QS of rumen microorganisms in beef cattle exposed to HS.
View Article and Find Full Text PDFAntioxidants (Basel)
February 2025
Department of Anaesthesiology, University Hospital of Split, 21000 Split, Croatia.
Cardiac surgery-related acute kidney injury (CS-AKI) is a decrease in kidney function after open-heart surgery, affecting up to 50% of patients. The pathophysiology of CS-AKI involves ischemia-reperfusion injury, inflammation, and oxidative stress. Ubiquinone is a potent antioxidant, and we hypothesized that it could decrease both the incidence and severity of CS-AKI.
View Article and Find Full Text PDFJCI Insight
November 2024
Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center and Vanderbilt University School of Medicine, Nashville, Tennessee, USA.
Urinary concentration is an energy-dependent process that minimizes body water loss by increasing aquaporin 2 (AQP2) expression in collecting duct (CD) principal cells. To investigate the role of mitochondrial (mt) ATP production in renal water clearance, we disrupted mt electron transport in CD cells by targeting ubiquinone (Q) binding protein QPC (UQCRQ), a subunit of mt complex III essential for oxidative phosphorylation. QPC-deficient mice produced less concentrated urine than controls, both at baseline and after type 2 vasopressin receptor stimulation with desmopressin.
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