Low Coenzyme Q10 Levels Research Articles

Evaluation of the Coenzyme Q10 and Some Biochemical Parameters in Patients with Ischemic Heart Disease.

Ischemic heart disease (IHD) is a common diagnosis and a leading cause of death in both males and females. It accounts for 30% of deaths worldwide, including 40% in high-income countries and approximately 28% in developing nations. Several cardiac markers have been used to diagnose and manage cardiovascular diseases. The Coenzyme Q10 (CoQ10) plays a potential role in the prevention and treatment of cardiovascular diseases by improving cellular bioenergetics. This study aimed to evaluate the role of CoQ10 and other biochemical parameters in IHD (angina pectoris and myocardial infarction). A case-control study was conducted at the Intensive Care Unit of Ibn-Sina Teaching Hospital and Al-Salam General Hospital in Nineveh Province, Iraq, for two months, from April 1 to June 1, 2022. It included 90 adult participants divided into case and control groups. The case group included 60 patients admitted to the Intensive Care Unit and diagnosed with IHD (myocardial infarction or angina pectoris), and the control group included 30 healthy participants matched in age and gender with the case group. Subsequent assay of C-reactive protein (CRP), creatine phosphokinase (CPK), troponin level, and serum CoQ10. In this study, 81.7% of patients in the case group were diagnosed with myocardial infarction. Means of serum lactate dehydrogenase (LDH), CRP, CPK, and troponin were significantly higher, while those of CoQ10 were significantly lower in the case group, compared to the controls. Statistically, a significant moderate negative correlation was detected between CoQ10 level and age. Moreover, significant weak correlations were observed between CoQ10 level and all serum LDH, CRP, and troponin levels. Patients with IHDs had considerably low serum levels of CoQ10, compared to the control group. The highest mean value of lipid profile, except for triglyceride, was observed in patients with IHD, compared to the control group. This explains the role that cholesterol compounds play in the progression of IHD. No significant correlations were found between CoQ10 with body mass index and CPK. The CoQ10 had a negative correlation with age, serum LDH, CRP, and troponin.

Coenzyme Q10 Levels Associated With Cognitive Functioning and Executive Function in Older Adults.

Brain deterioration with age is associated with inflammation and oxidative stress that result in structural and functional changes. Recent studies have indicated that coenzyme Q10 (CoQ10) is associated with neurological oxidative stress and cognitive impairment. Studies with older people have shown a relationship between neurodegenerative diseases and CoQ10 levels. However, no studies have analyzed the relationship between CoQ10 and cognitive functioning in older adults. The aim of this study was to analyze the association between CoQ10 and cognitive functioning in an older adult sample, controlling for other factors that may influence aging, such as the level of physical activity and nutritional status. The sample consisted of 64 older adults aged 65-99 years (76.67 ± 8.16 years), among whom 48 were women (75%). The participants were recruited among those who attended community centers to voluntarily participate in leisure activities. According to previous studies, physical activity and nutritional status are positively associated with cognitive functioning. However, the main finding of this study was that plasma CoQ10, controlling for other measures, was significantly associated with cognitive functioning and executive function. The current findings suggest that a decline in cognitive capacities may be related to reduced antioxidant defenses, as reflected by low CoQ10 levels in older adults.

Network Analyses Links Epicardial Fat Metabolism to Human Coronary Heart Disease

The generation of tissue-specific integrated networks (INs) through the merging of transcriptional regulatory, protein-protein interaction and genome-scale metabolic networks allows for a rigorous investigation of the biological processes that are altered in cardiovascular disease (CVD) subjects. We developed INs for left ventricle (LV) and visceral adipose tissue (omentum) of healthy subjects and compared these with LV and epicardial fat INs for CVD subjects. We investigated changes in co-regulation for enzymes in pathways that are known to be associated with the development and progression of CVD for each tissue type. Global gene expression changes between healthy and CVD subjects were determined for both tissue types and reporter metabolite analyses were performed. The application and combination of these different network approaches enabled us to associate low levels of Coenzyme Q10 with cardiac remodelling in the LV. We also found increasing homocysteine levels with dysregulation of the adipocytokine pathway in the epicardial fat of CVD subjects and validated our predictions by detecting increased plasma metabolite levels associated with homocysteine in CVD subjects.

Prospective study of plasma levels of coenzyme Q10 and lung cancer risk in a low-income population in the Southeastern United States.

BackgroundCoenzyme Q10 (CoQ10) is a ubiquitous molecule in living organisms serving as a cofactor in energy production. Epidemiological studies have reported low CoQ10 levels being associated with an increased risk of various cancers. We conducted the first study to evaluate the association of CoQ10 concentrations with lung cancer risk.MethodsA nested case‐control study including 201 lung cancer cases and 395 matched controls from the Southern Community Cohort Study was conducted. Plasma CoQ10 levels were measured using high‐performance liquid chromatography with photo‐diode array detection. Conditional logistic regression models were applied to estimate odds ratios (ORs) and 95% confidence intervals (CIs) for the association between plasma CoQ10 levels and lung cancer risk.ResultsPlasma CoQ10 concentration was inversely associated with the risk of lung cancer. After adjusting for age, sex, race, and socioeconomic status, the OR (95% CI) comparing the third to first tertile was 0.57 (0.36–0.91, P for trend = 0.02). Further adjustments for smoking, alcohol, chronic obstructive pulmonary disease, and body mass index attenuated the point estimate slightly (OR = 0.60, 95% CI = 0.34–1.08, P for trend = 0.11), comparing third to first tertiles. Stratified analyses identified a significant inverse association between plasma CoQ10 levels and lung cancer risk in current smokers, but not in former/never smokers. The association was more evident in cases who were diagnosed within 1 year of blood draw than in cases diagnosed after 1 year.ConclusionsLow plasma CoQ10 was significantly associated with increased lung cancer risk, particularly among current smokers. The stronger association seen shortly following the blood draw suggests that CoQ10 may be related to disease progression.

Effect of coenzyme Q10 supplementation on oxidative stress and clinical outcomes in patients with low levels of coenzyme Q10 admitted to the intensive care unit.

Today, trauma is known to be the third leading cause of death in most countries. Studies have demonstrated below-normal plasma levels of antioxidants in trauma patients. The present study aimed to assess the efficacy of Coenzyme Q10 (CoQ10) on oxidative stress, clinical outcomes and anthropometrical parameters in traumatic mechanical ventilated patients admitted to the intensive care unit. Patients were randomised to receive sublingual CoQ10 (400 mg/d) or placebo for 7 d. Primary and secondary outcomes were measured at the baseline and end of the study. We enrolled forty patients for this trial: twenty in the CoQ10 group and twenty in the placebo group. There was not any significant difference in the baseline variables (P > 0⋅05). At the end of the study, CoQ10 administration caused a considerable reduction in the Malondialdehyde (MDA) and Interleukin 6 (IL-6) concentrations (P < 0⋅001), Glasgow Coma Score (GCS; P = 0⋅02), ICU and hospital length of stay and mechanical ventilation (MV) duration (P < 0⋅001). We found that CoQ10 administration could increase Fat-Free Mass (P < 0⋅001) (FFM; P = 0⋅04), Skeletal Muscle Mass (SMM; P = 0⋅04) and Body Cell Mass (BCM) percent (P = 0⋅03). There was not any significant difference in other factors between the two groups (P > 0⋅05). CoQ10 administration has beneficial effects on patients with traumatic injury and has no side effects. However, since the possibility of the type II error was high, the outcomes on the duration of MV, ICU stay and hospital stay, and GCS may very well be false positives.

Low coenzyme Q10 levels in patients with acute cardiovascular disease are associated with long-term mortality

Coenzyme Q10 (CoQ10) plays a potential role in the prevention and treatment of cardiovascular disease through improved cellular bioenergetics. Critical illness in the intensive care unit has been reported to be associated with decreased circulating CoQ10 levels, and we previously demonstrated the association of low CoQ10 levels with in-hospital mortality. However, the association of CoQ10 with the acute phase of cardiovascular disease and long-term mortality remains unclear. We enrolled 242 consecutive patients with cardiovascular disease admitted to the coronary care unit of Juntendo University Hospital to investigate the association between long-term mortality and serum CoQ10 levels. During a mean follow-up of 3.2years, 58 patients died. The mean serum CoQ10 levels were significantly lower in the non-survivors than in the survivors (0.48 ± 0.27 vs. 0.58 ± 0.38mg/L; p = 0.035). Compared with the patients with above-median CoQ10 levels (0.46mg/L), the cumulative incidence of all-cause mortality was significantly higher in those with lower CoQ10 levels (p = 0.025). Multivariate Cox regression analysis further demonstrated that lower CoQ10 levels were associated with poor prognosis. Low serum CoQ10 levels during the acute phase of cardiovascular diseases were associated with long-term mortality in patients, suggesting the utility of low serum CoQ10 levels as a predictor and potential therapeutic target.

Clinical Presentation, Genetic Etiology, and Coenzyme Q10 Levels in 55 Children with Combined Enzyme Deficiencies of the Mitochondrial Respiratory Chain

To evaluate the clinical symptoms and biochemical findings and establish the genetic etiology in a cohort of pediatric patients with combined deficiencies of the mitochondrial respiratory chain complexes. Clinical and biochemical data were collected from 55 children. All patients were subjected to sequence analysis of the entire mitochondrial genome, except when the causative mutations had been identified based on the clinical picture. Whole exome sequencing/whole genome sequencing (WES/WGS) was performed in 32 patients. Onset of disease was generally early in life (median age, 6weeks). The most common symptoms were muscle weakness, hypotonia, and developmental delay/intellectual disability. Nonneurologic symptoms were frequent. Disease causing mutations were found in 20 different nuclear genes, and 7 patients had mutations in mitochondrial DNA. Causative variants were found in 18 of the 32 patients subjected to WES/WGS. Interestingly, many patients had low levels of coenzyme Q10 in muscle, irrespective of genetic cause. Children with combined enzyme defects display a diversity of clinical symptoms with varying age of presentation. We established the genetic diagnosis in 35 of the 55 patients (64%). The high diagnostic yield was achieved by the introduction of massive parallel sequencing, which also revealed novel genes and enabled elucidation of new disease mechanisms.

Serum Coenzyme Q10 Levels are Decreased in Critically-Ill Septic Patients: Results From a Preliminary Study.

The increased oxidative stress resulting from the inflammatory responses in sepsis initiates changes in mitochondrial function which may result in organ damage, the most common cause of death in the intensive care unit (ICU). Deficiency of coenzyme Q10 (CoQ10), a key cofactor in the mitochondrial respiratory chain, could potentially disturb mitochondrial bioenergetics and oxidative stress, and may serve as a biomarker of mitochondrial dysfunction. Hence, we aimed to investigate in initially non-septic patients whether CoQ10 levels are decreased in sepsis and septic shock compared to ICU admission, and to evaluate its associations with severity scores, inflammatory biomarkers, and ICU outcomes. Observational retrospective analysis on 86 mechanically-ventilated, initially non-septic, ICU patients. CoQ10 was sequentially measured on ICU admission, sepsis, septic shock or at ICU discharge. CoQ10 was additionally measured in 25 healthy controls. Inflammatory biomarkers were determined at baseline and sepsis. On admission, ICU patients who developed sepsis had lower CoQ10 levels compared to healthy controls (0.89 vs. 1.04 µg/ml, p < 0.05), while at sepsis and septic shock CoQ10 levels decreased further (0.63 µg/ml; p < 0.001 and 0.42 µg/ml; p < 0.0001, respectively, from admission). In ICU patients who did not develop sepsis, admission CoQ10 levels were also lower than healthy subjects (0.81 µg/ml; p < 0.001) and were maintained at the same levels until discharge. CoQ10 levels in critically-ill patients are low on ICU admission compared to healthy controls and exhibit a further decrease in sepsis and septic shock. These results suggest that sepsis severity leads to CoQ10 depletion.

Coenzyme Q10 in association with metabolism-related AMPK/PFKFB3 and angiogenic VEGF/VEGFR2 genes in breast cancer patients.

Low levels of coenzyme Q10 (CoQ10) have been reported in the circulation of patients with breast cancer, particularly in metastatic features. Our objective was to study the correlation between plasma levels of CoQ10 and the tumoral expression levels of AMPK, PFKFB3, VEGF, and VEGFR2. This study was a part of consecutive case series conducted on 100 women with newly diagnosed invasive ductal breast carcinoma, with an age range of 30-60years. Plasma levels of CoQ10 were measured using HPLC coupled toan UV detector. The expression levels were quantified using quantitative real-time PCR. Structural equation modeling (SEM) was applied to generate pathways describing gene-to-gene inter-correlations. Using SEM identified AMPK expression to contribute positively to VEGF-A/VEGFR2 ratio (coefficient b = 0.64, P < 0.001). The VEGFR2 expression positively correlated with tumor size (coefficient b = 0.31, P < 0.001). A linear correlation between expression levels of AMPK and PFKFB3 was observed (rAdj = - 0.273, P = 0.02). Similarly, VEGF-A was correlated with VEGFR2 (rAdj = 0.698, P < 0.001). There were inverse significant correlations between CoQ10 and the fold changes of AMPK (rAdj = - 0.276, P = 0.030), VEGF-A (rAdj = - 0.319, P = 0.011) and VEGFR2 (rAdj = - 0.262, P = 0.045). The correlation between CoQ10 and the fold changes of PFKFB3 was significantly progesterone receptor (PR)dependent (rAdj = - 0.284, P = 0.041). PlasmaCoQ10 was correlated with VEGF-A in hormone receptor-dependent mode (ER + : rAdj = - 0.286, P = 0.032 and PR + : rAdj = - 0.313, P = 0.025). Our findings could provide new insights suggesting CoQ10 can inversely correlate to the expression levels of VEGF-A/VEGFR2 as angiogenic factors and AMPK/PFKFB3 as biomarkers for tumoral glycolysis, especially in a hormone receptor-dependent manner to possibly prevent theprogression of breast carcinogenesis.

Abstract 595: Circulating levels of coenzyme Q10 and lung cancer risk

Abstract Background: Coenzyme Q10 (CoQ10) is an ubiquitous molecule in living organisms that serves as a cofactor in energy production via the electron-transport chain (ETC). Research has previously shown that deficiencies in CoQ10 can result in a variety of detrimental outcomes including cardiovascular disease and neurological disorders. More recently, epidemiological studies have reported low CoQ10 levels being associated with increased risk of breast, ovarian, and skin cancers. However, studies exploring the link between CoQ10 and lung cancer risk are largely lacking. Methods: A nested case-control study including 201 lung cancer cases and 395 matched controls from the Southern Community Cohort Study (SCCS) was conducted. Plasma CoQ10 levels were measured using high-performance liquid chromatography (HPLC). A conditional logistic regression model was applied to estimate the odds ratio (OR) and 95% confidence interval (CI) for the association between plasma CoQ10 concentrations and lung cancer risk. CoQ10 level was categorized by tertile distribution among controls. Results: Plasma CoQ10 concentration was inversely associated with risk of lung cancer. Compared to the first tertile, the ORs (95% CI) were 0.82 (0.52-1.31) and 0.57 (0.34-0.96) for the second and third tertile, respectively, (P for trend=0.038), after adjusting for age, smoking status and pack-years of smoking. Additional adjustment for alcohol consumption, education, income, history of COPD and BMI slightly attenuated the association (OR=0.60, 95% CI=0.34-1.08, comparing third to first tertile). No notable difference was found between African Americans and European Americans. Stratified analysis identified a significant association between CoQ10 and lung cancer in current smokers (OR=0.47, 95% CI=0.26-0.87, third vs. first tertile), but not in former/never smokers (OR=0.84, 95% CI=0.35-2.03). The significant associations between CoQ10 and lung cancer risk were more evident in cases who were diagnosed within one year of blood draw (OR=0.17, 95% CI=0.04-0.73, third vs. first tertile, P for trend=0.023) than in cases diagnosed after one year following blood draw (OR=0.72, 95% CI=0.36-1.41). Conclusions: Findings from this study suggest that Coenzyme Q10 may be a potential marker for lung cancer, although the stronger effect shortly after diagnosis indicates the association may be related to dietary or other changes associated with the cancer diagnosis rather than CoQ10 being a causal risk biomarker. Further studies exploring the link between CoQ10 and lung cancer risk are necessary to better understand the role of CoQ10 in lung carcinogenesis. Citation Format: Chris Shidal, Hyung-Suk Yoon, Wei Zheng, Jie Wu, Adrian A. Franke, William J. Blot, Xiao-Ou Shu, Qiuyin Cai. Circulating levels of coenzyme Q10 and lung cancer risk [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2019; 2019 Mar 29-Apr 3; Atlanta, GA. Philadelphia (PA): AACR; Cancer Res 2019;79(13 Suppl):Abstract nr 595.

CoQ10 and Aging.

The aging process includes impairment in mitochondrial function, a reduction in anti-oxidant activity, and an increase in oxidative stress, marked by an increase in reactive oxygen species (ROS) production. Oxidative damage to macromolecules including DNA and electron transport proteins likely increases ROS production resulting in further damage. This oxidative theory of cell aging is supported by the fact that diseases associated with the aging process are marked by increased oxidative stress. Coenzyme Q10 (CoQ10) levels fall with aging in the human but this is not seen in all species or all tissues. It is unknown whether lower CoQ10 levels have a part to play in aging and disease or whether it is an inconsequential cellular response to aging. Despite the current lay public interest in supplementing with CoQ10, there is currently not enough evidence to recommend CoQ10 supplementation as an anti-aging anti-oxidant therapy.

Liver Antioxidants in Relation to Beak Morphology, Gizzard Size and Diet in the Common Eider Somateria mollissima.

Antioxidants in the liver are particularly abundant in capital breeders that rely on stored resources for egg production. Capital breeders like eider (hereafter common eider) Somateria mollissima have disproportionately large livers with low levels of coenzyme Q10 when compared to other bird species. Concentrations of total carotenoids and vitamin E in the livers of eiders were smaller than predicted for similarly sized bird species. Eiders with high body condition estimated as body mass relative to skeletal body size had high levels of total carotenoids and low levels of coenzyme Q10. The concentration of total carotenoids per gram of liver increased with age, and vitamin E and total carotenoids accumulated during the winter onwards from February to peak at the start of incubation in April. Total vitamin E, total carotenoids, and coenzyme Q10 per gram of liver decreased with increasing beak volume. The size of the empty gizzard increased with increasing liver mass but decreased with total carotenoids and coenzyme Q10. The main components of the diet were blue mussels Mytilus edulis (40%), draft whelk Nassarius reticulatus (27%), and periwinkle Littorina littorea (10%). The concentration of vitamin E increased with the number of razor clams Ensis sp. and draft whelks in the gizzard and the concentration of total carotenoids increased with the number of beach crabs Carcinus maenas. These observations are consistent with the hypothesis that eiders are limited in their levels of antioxidants through food limitation. Furthermore, they imply that diet and morphological characters involved in food acquisition and processing are important determinants of the level of antioxidants in the liver.

Brain-derived neurotrophic factor and coenzyme Q10 levels in blood of children with learning disorder

Background Learning disorder (LD) is manifested by significant difficulties in the acquisition and use of reasoning, reading, writing, or mathematical abilities, despite average intelligence and proper education. Its etiological factors were suggested to be related to neurodevelopmental alterations. Measurement of the levels of brain-derived neurotrophic factor (BDNF) and coenzyme Q10 (CoQ10) was targeted in children with LD in comparison with typically developing (TD) ones. Materials and methods This study included 82 Egyptian Arabic-speaking children matched for age and sex and socioeconomic status, comprising 42 with specific LD (group I) and 40 TD children (group II). All participants were subjected to clinical and full neurological examination after reporting a full medical history. Furthermore, LD group was subjected to Stanford–Binet intelligence scale, dyslexia assessment test, and phonological awareness test, which evaluates cognitive and learning aptitudes. The levels of BDNF and CoQ10 were determined in serum by enzyme-linked immunosorbent assay. Results All children with LD obtained a score of 1 or more as at-risk quotient by the dyslexia assessment test, which indicated a specific reading disorder. The BDNF and CoQ10 levels in the LD group were significantly less than those in the TD group. No correlations were found between the measured markers and each other or between them and the measured factors of the used tests. Conclusion The detected low levels of BDNF and CoQ10 in children with specific LD with impairment in reading would be suspected to be related to etiological or exaggerating factors for the deficits in such children.

Low circulating coenzyme Q10 during acute phase is associated with inflammation, malnutrition, and in-hospital mortality in patients admitted to the coronary care unit.

Coenzyme Q10 (CoQ10) has a potential role in the prevention and treatment of heart failure through improved cellular bioenergetics. In addition, it has antioxidant, free radical scavenging, and vasodilatory effects that may be beneficial. Although critical illness in intensive care unit is associated with decreased circulating CoQ10 levels, the clinical significance of CoQ10 levels during acute phase in the patients of cardiovascular disease remains unclear. We enrolled 257 consecutive cardiovascular patients admitted to the coronary care unit (CCU). Serum CoQ10 levels were measured after an overnight fast within 24h of admission. We examined the comparison of serum CoQ10 levels between survivors and in-hospital mortalities in patients with cardiovascular disease. Serum CoQ10 levels during the acute phase in patients admitted to the CCU had similar independent of the diagnosis. CoQ10 levels were significantly lower in patients with in-hospital mortalities than in survivors (0.43±0.19 vs. 0.55±0.35mg/L, P=0.04). In patients admitted to the CCU, CoQ10 levels were negatively associated with age and C-reactive protein levels, and positively associated with body mass index, total cholesterol, and high-density lipoprotein cholesterol levels. Low CoQ10 levels correlated with low diastolic blood pressure. Multivariate logistic regression analysis demonstrated that low CoQ10 levels were an independent predictor of in-hospital mortality. Low serum CoQ10 levels during acute phase are significantly associated with cardiovascular risk and in-hospital mortality in patients admitted to the CCU.

Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum.

In familial and sporadic multiple system atrophy (MSA) patients, deficiency of coenzyme Q10 (CoQ10) has been associated with mutations in COQ2, which encodes the second enzyme in the CoQ10 biosynthetic pathway. Cerebellar ataxia is the most common presentation of CoQ10 deficiency, suggesting that the cerebellum might be selectively vulnerable to low levels of CoQ10 To investigate whether CoQ10 deficiency represents a common feature in the brains of MSA patients independent of the presence of COQ2 mutations, we studied CoQ10 levels in postmortem brains of 12 MSA, 9 Parkinson disease (PD), 9 essential tremor (ET) patients, and 12 controls. We also assessed mitochondrial respiratory chain enzyme activities, oxidative stress, mitochondrial mass, and levels of enzymes involved in CoQ biosynthesis. Our studies revealed CoQ10 deficiency in MSA cerebellum, which was associated with impaired CoQ biosynthesis and increased oxidative stress in the absence of COQ2 mutations. The levels of CoQ10 in the cerebella of ET and PD patients were comparable or higher than in controls. These findings suggest that CoQ10 deficiency may contribute to the pathogenesis of MSA. Because no disease modifying therapies are currently available, increasing CoQ10 levels by supplementation or upregulation of its biosynthesis may represent a novel treatment strategy for MSA patients.

Coenzyme Q10 and oxidative stress, the association with peripheral sensory neuropathy and cardiovascular disease in type 2 diabetes mellitus

ObjectiveOur study aimed to explore associations between metabolic control, oxidative stress and coenzyme Q10 (CoQ10) in relation to diabetes complications in a representative population of type 2 diabetes. Research design and methodsA geographic cohort of 156 subjects was recruited. Serum concentrations of CoQ10 and vitamin E were measured by HPLC. ROS was determined by free oxygen radicals testing (FORT). Glutaredoxin (Grx) activity, oxidized LDL cholesterol (oxLDLc), high sensitive CRP (hsCRP), HbA1c, urine albumin, serum creatinine, serum cystatin C, and plasma lipids were assayed with routine laboratory protocols. ResultsSerum CoQ10 was higher than in nondiabetics. HbA1c, fP-glucose, hyperlipidemia, inflammation (hsCRP), and increased BMI were associated with signs of oxidative stress as increased levels of FORT, Grx activity and/or increased levels of oxLDLc Oxidative stress was found to be strongly correlated with prevalence of cardiovascular disease (CVD) and peripheral sensory neuropathy (PSN). In both gender groups there were positive correlations between CoQ10 and oxLDLc, and between BMI and the ratio CoQ10/chol. Grx activity was inversely correlated to oxLDLc and CoQ10. Women with CVD and PSN had higher waist index, oxLDLc, and FORT levels compared to men but lower CoQ10 levels. Men had worse kidney function and lower vitamin E. Multiple regression analysis showed increased levels of CoQ10 to be significantly correlated with increased levels of cholesterol, triglycerides, vitamin E, fB-glucose and BMI. ConclusionsHyperlipidemia, hyperglycemia and inflammation were associated with oxidative stress, which was correlated to the prevalence of diabetes complications. CoQ10 was increased in response to oxidative stress. There were gender differences in the risk factors associated with diabetes complications.

OP44 – 2555: The association of coenzyme Q10 deficiencies and respiratory chain disorders in South African patients

Objective Although coenzyme Q10 (CoQ10) deficiencies are heterogeneous it is often associated with mitochondrial respiratory chain disorders (RCD), specifically complex II+III (CII+II) deficiencies. The aim of this study was to investigate the association of defects in the biosynthesis of CoQ10 and associated RCD in South African patients. Methods A method to quantify CoQ10 in muscle was developed and standardized using isotope-dilution liquid chromatography-tandem mass spectrometry (LC-MS-MS). The CoQ10 quantification was then performed on 155 muscle specimens of patients who clinically qualified for respiratory chain enzymes analyses. Of these patients, 76 had confirmed RCD of which 29 patients had CII+III deficiencies. Ion Torrent next generation sequencing (NGS) was performed on 24 patients, which included nine patients with decreased and 15 with normal CoQ10 levels. Eighteen targeted genes involved in primary as well as secondary CoQ10 deficiencies were sequenced. Results CoQ10 was deficient in nine patients of which eight patients had CII+III deficiency. Race, age and gender did not have any influence on the CoQ10 levels. A total of 16 possible disease-causing variants were identified. Three novel compound heterozygous variants in three patients (two with deficient CoQ10 levels) were found in the ETFDH, COQ6 and COQ7 genes respectively. Protein analyses supported pathogenicity of the variants in the ETFDH and COQ6 genes. Conclusion Only RCD patients with confirmed CII+III enzyme deficiencies had statistically significant low muscle CoQ10 levels and at least two patients had novel compound heterozygous mutations in the ETFDH and COQ6 genes.

Effect of Coenzyme Q10 on Th1/Th2 Paradigm in Females with Idiopathic Recurrent Pregnancy Loss.

Recurrent pregnancy loss is characterized by predominant Th1-type immunity and increased reactive oxygen species. Low levels of Coenzyme Q10 are found in the plasma of RPL as compared to healthy pregnant females. Our aim was to investigate whether in vitro supplementation of PBMCs from such females with CoQ10 could change the observed Th1 bias. PBMCs were isolated from 20 RPL pregnant and non-pregnant females and 16 healthy pregnant females and incubated with CoQ10 in in vitro conditions. Phenotyping of Th1, Th2, and Th17 cells was performed by flow cytometry. Cytokine levels were determined by ELISA. PBMCs treated with CoQ10 showed significantly decreased percentage of Th1 cells (P < 0.005) in pregnant females with history of RPL than in the untreated ones. Also, levels of IFN-γ and TNF-α were significantly decreased in the culture supernatant of treated PBMCs from RPL. DCFDA staining showed significantly reduced production of ROS in the treated PBMCs in RPL females. CoQ10 was effective in maintaining the immune homeostasis by reducing the proportion of IFN-γ-producing T cells and proinflammatory cytokine levels in the RPL pregnant females. This property could be attributed to the capability of CoQ10 in reducing oxidative stress by decreasing ROS production.

New analytical strategies applied to the determination of Coenzyme Q10 in biological matrix.

In the last few years the importance of Coenzyme Q10 (CoQ10) determination has gained clinical relevance. CoQ10 is a redox-active, lipophilic substance integrated in the mitochondrial respiratory chain which acts as an electron carrier for the production of cellular energy. In addition, it is recognized as a primary regenerating antioxidant playing an intrinsic role against oxidative damage. There are some reports of low CoQ10 levels in a number of disorders, such as cancer, muscular, neurodegenerative, cardiological, and reproductive diseases. Therefore, it is a priority to develop analytical methodologies for evaluating CoQ10 in matrices of greater importance for the correct diagnosis of diseases, simple enough to be used in routine clinical laboratories. In this chapter two recently developed techniques, capillary electrophoresis and microHPLC, for the analysis of CoQ10 in biological matrices, are studied.

THU0305 Effect of Oral Coenzyme Q10 Supplementation on Clinical Symptoms and Oxidative Stress in Fibromyalgia Patients: A Randomized Trial

BackgroundFibromyalgia (FM) is a common and complex clinical condition characterized by chronic widespread musculoskeletal pain, fatigue and muscle tenderness. The pathophysiology of FM is still not well elucidated. In recent...