Exploring Plasma Coenzyme Q10 Status in Paediatric Dyslipidaemia

Author:

Minguez Beatriz1ORCID,de Los Santos Mariela1,Garcia-Volpe Camila1,Molera Cristina1,Paredes-Fuentes Abraham J.2ORCID,Oliva Clara2ORCID,Arias Angela3,Rodriguez-Gonzalez Helena3ORCID,Yubero Delia4,Tondo Mireia56ORCID,Santos-Ocaña Carlos78ORCID,Meavilla Silvia1,Artuch Rafael38ORCID

Affiliation:

1. Gastroenterology and Nutrition Department, Hospital Sant Joan de Déu, 08950 Barcelona, Spain

2. Division of Inborn Errors of Metabolism-IBC, Biochemistry and Molecular Genetics Department, Hospital Clínic de Barcelona, 08036 Barcelona, Spain

3. Clinical Biochemistry Department, Institut de Recerca Sant Joan de Déu, 08950 Barcelona, Spain

4. Genetic Department, Hospital Sant Joan de Déu, 08950 Barcelona, Spain

5. Department of Biochemistry, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute (IIB) Sant Pau, 08041 Barcelona, Spain

6. Centre of Biomedical Investigation Network for Diabetes and Metabolic Diseases (CIBERDEM), 28029 Madrid, Spain

7. Departamento de Fisiología, Anatomía y Biología Celular, Centro Andaluz de Biología del Desarrollo, Universidad Pablo de Olavide, 41013 Sevilla, Spain

8. U703-U729 CIBERER, Instituto de Salud Carlos III, 28029 Madrid, Spain

Abstract

Coenzyme Q10 (CoQ) is a ubiquitous lipid with different biological functions. In blood, there is a close relationship between CoQ status and cholesterol, which strongly supports the study of both molecules simultaneously. The objective of this study was to evaluate plasma CoQ, lipoprotein concentrations and CoQ/Chol ratio in a cohort of paediatric patients with different types of dyslipidaemias. A total of 60 paediatric patients were recruited (age range: 7 months–18 years), including 52 with different types of hypercholesterolemia, 2 with isolated hypertriglyceridemia and 6 with hypobetalipoproteinemia. Plasma CoQ was analysed by HPLC with electrochemical detection, and lipoprotein and cholesterol concentrations by standard automated methods. The lowest CoQ values were detected in patients with hypobetalipoproteinemia and in two cases of liver cirrhosis. Mean CoQ values were significantly higher in hypercholesterolemic patients compared to controls (average values 1.07 µmol/L and 0.63 µmol/L) while the CoQ/cholesterol ratio did not show differences (170 vs. 163, respectively). Mean CoQ values were significantly lower in the group of patients with hypobetalipoproteinemia compared to controls (mean CoQ values of 0.22 µmol/L vs. 0.63 µmol/L, respectively), while those of CoQ/cholesterol did not show differences. Pearson’s correlation test showed a positive correlation between the CoQ and cholesterol values (r = 0.565, p < 0.001) and between the CoQ and the LDL cholesterol values (r = 0.610, p < 0.001). Our results suggest that it is advisable to analyse plasma CoQ and cholesterol concentrations in patients with hypobetalipoproteinemia and hypercholesterolemia associated with liver damage.

Publisher

MDPI AG

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