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Coenzyme Q10 and L-Carnitine Disturbances in Children with Mitochondrial Diseasesстатья Электронная публикация Глава в книге
- Авторы: Nikolaeva E.А., Mamedov I.S., Zolkina I.V.
- Сборник: Mitochondrial and Brain Disorders
- Глава в коллективной монографии
- Год издания: 2019
- Первая страница: 1
- Последняя страница: 14
- DOI: 10.5772/intechopen.87950
- Аннотация: Coenzyme Q10 (CoQ10) and L-carnitine are very important biologically activecompounds involved in energy metabolism. L-carnitine and coenzyme Q10 disturbancesin mitochondrial diseases require the correction. Patients and methods: Thelevels of coenzyme Q10 and L-carnitine (total carnitine, free carnitine, and acylcarnitines)were determined in children with mitochondrial diseases (25 childrenand 16 children, respectively). High-performance liquid chromatography with UVdetection (chromatograph Shimadzu Nexera LC-30) and chromatography-massspectrometry (Agilent 6410 QQQ , USA) were used. As an additional parameter ofpossible coenzyme Q10 and carnitine insufficiency, the coenzyme Q10/cholesterolratio and acylcarnitines/free carnitine ratio were calculated. Results: A significantlylow ratio of coenzyme Q10/cholesterol in children with mitochondrial diseaseswas revealed—0.10 ± 0.01 vs. 0.19 ± 0.01 in the control group (p < 0.001). A lowerabsolute level of coenzyme Q10 and tendency toward a more pronounced decreasein the Q10/cholesterol ratio in older patients (6–16 years) were shown. The freecarnitine blood level was within the normal range and averaged at 29.8 ± 2.6 μmol/l;however, the level was lower than that in the control group (44 ± 5.2 μmol/l,p < 0.05). A pronounced significant increase in the acylcarnitines/free carnitineratio was determined—1.5 ± 0.05 (the normal range < 0.6).
- Добавил в систему: Мамедов Ильгар Салехович