In a 2021 study, patients with hereditary hemochromatosis were observed to have significantly lower plasma concentrations of Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More [Martinefski 2021].
Hereditary hemochromatosis is a genetic disorder that causes the body to build up too much iron in various organs including the heart and liver. The iron overload is toxic and can damage tissues and organs and lead to liver disease [CDC 2020].
The CDC reports that one-third of one percent of non-Hispanic Caucasians in the US has hereditary hemochromatosis. The rates are somewhat lower in other races and ethnic groups. It is more common for men to develop complications, often at an earlier age. Approximately one in ten men with hereditary hemochromatosis will develop severe liver disease.
Plasma CoQ10 Levels in Hereditary Hemochromatosis Patients
Martinefski et al. [2021] analyzed the plasma CoQ10 concentrations in 38 patients (32 males, 6 female) newly diagnosed with hereditary hemochromatosis (less than 4 weeks) who had not yet been treated for the disease. The patients ranged in age from 23 to 69 years.
The researchers compared the patients’ plasma CoQ10 levels with the plasma CoQ10 levels in 25 healthy controls (22 male, 3 female) aged 24 to 68 years.
The patients had plasma CoQ10 levels of 0.27 mg/L on average, very low plasma CoQ10 levels.
For the controls, the mean plasma CoQ10 level was 0.61 mg/L, still not very high but within the reference range for unsupplemented adults.
There is, of course, very considerable inter-individual variability in CoQ10 levels, and the samples in this study were small: 38 patients and 25 controls.
Coenzyme Q10 and Oxidative Stress in Hereditary Hemochromatosis
The researchers regarded the decreased plasma CoQ10 levels as evidence of an imbalance between oxidative stress and antioxidantAntioxidants are substances that protect the cells and lipoproteins against the harmful effects of free radicals. They are substances that prevent the oxidation of other molecules and compounds. There are two broad categories of antioxidants: enzymatic and non-enzymatic. Non-enzymatic antioxidants are substances like Coenzyme Q10, vitamin C, vitamin E, glutathione, and various carotenoids. Prominent enzymatic antioxidants include catalase, glutathione peroxidase,... More defenses in the hereditary hemochromatosis patients [Martinefski 2021].
They suggested that the plasma CoQ10 depletion may have resulted from increased transfer of Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More from the blood to the cells and tissues to counteract the increased oxidative stress caused by the disease [Martinefski 2021].
The depletion of Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More, in turn, would adversely affect the need of the heart muscle cells (and cells in other organs requiring energy) for cellular energy production [Martinefski 2021].
Note: Combined daily supplementation of community living senior citizens with Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More and seleniumSelenium (symbol Se, atomic number 34) is a trace element that is an essential nutrient and an essential component of some of the most important antioxidants in the body, in particular the selenoproteins glutathione peroxidase, thioredoxin reductase, and selenoprotein P. Selenium is involved in the optimal functioning of the immune system. Professor Alehagen has pointed out that there exists a... More for four years in the KiSel-10 StudyIn the KiSel-10 study, 443 Swedish citizens aged 70 to 88 received either a combined daily supplementation of high-selenium yeast and Coenzyme Q10 or matching placebos. The elderly Swedish citizens who received the active treatment of selenium and Coenzyme Q10 had significantly reduced risk of death from heart disease, significantly better heart function as recorded on echocardiograms, and significantly lower... More has shown beneficial effects of the supplementation on bio-markers of oxidative stress and inflammationInflammation is an immune system response to an injury to cells or tissues. It is the body’s attempt to defend against invaders such as bacteria andviruses and to mend the damage done by invaders. Typically, inflammation manifests itself in the form of fever and swelling, in the swarming of white blood cells and the release of cytokines to fight against... More [Alehagen 2015].
CoQ10 Levels Relative to Age and Lipid Metabolism
Martinefski et al [2021] knew that plasma and tissue CoQ10 levels tend to peak when humans are in their 20s and then to decline because of decreasing bio-synthesis [Kalén 1989].
To avoid any confounding by the age parameter, the researchers used an age—matched group experimental design.
The researchers knew, moreover, that Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More in the blood is incorporated to a very considerable extent into plasma lipoproteinsLipoproteins are particles comprised of protein and lipids, with a core of triglycerides and cholesterol esters and an outer layer of phospholipids. Lipoproteins transport lipids through the bloodstream. LDL-lipoproteins carry cholesterol from the liver to the body tissues. HDL- lipoproteins carry cholesterol back to the liver from the body tissues. LDL-lipoproteins also bind and transport Coenzyme Q10 molecules, predominately in... More. Accordingly, they normalized the plasma CoQ10 results to total cholesterolCholesterol is one of the major fat-soluble compounds that is found in animal plasma membranes. It is necessary for life and is found throughout the body. It is carried from the liver to the tissues where it is needed by lipoproteins of which it is a component. Much of the cholesterol is transported in Low density lipoproteins (LDL). High levels... More plasma levels. No differences were observed [Martinefski 2021].
Low CoQ10 Levels and Liver Disease
The researchers point to studies that show that Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More as a lipid-soluble antioxidantAntioxidants are substances that protect the cells and lipoproteins against the harmful effects of free radicals. They are substances that prevent the oxidation of other molecules and compounds. There are two broad categories of antioxidants: enzymatic and non-enzymatic. Non-enzymatic antioxidants are substances like Coenzyme Q10, vitamin C, vitamin E, glutathione, and various carotenoids. Prominent enzymatic antioxidants include catalase, glutathione peroxidase,... More is important to the control of iron-induced lipid peroxidationOxidation is the chemical reaction of a substance with oxygen. Peroxidation is the extreme form of oxidation that results from free radicals’ stealing electrons from lipids, either in the cell membranes or in the lipoproteins. The final product of lipid peroxidation is highly reactive malondialdehyde, a bio-marker for oxidative stress and damage. More and cell death called ferroptosis. They conclude that the CoQ10 deficiency in hereditary hemochromatosis patients could be an important potential mechanism in the development of iron-induced liver disease [Martinefski 2021].
Vitamin E Levels Also Low in Hereditary Hemochromatosis Patients
Martinefski et al. [2021] also observed lower levels of Vitamin EVitamin E is a fat-soluble vitamin that plays an important role as an antioxidant in the body. Vitamin E neutralizes harmful free radicals. Coenzyme Q10 regenerates Vitamin E after the vitamin E has neutralized free radicals. More in the plasma of the hereditary hemochromatosis patients.
Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More is known to regenerate vitamin EVitamin E is a fat-soluble vitamin that plays an important role as an antioxidant in the body. Vitamin E neutralizes harmful free radicals. Coenzyme Q10 regenerates Vitamin E after the vitamin E has neutralized free radicals. More as an antioxidantAntioxidants are substances that protect the cells and lipoproteins against the harmful effects of free radicals. They are substances that prevent the oxidation of other molecules and compounds. There are two broad categories of antioxidants: enzymatic and non-enzymatic. Non-enzymatic antioxidants are substances like Coenzyme Q10, vitamin C, vitamin E, glutathione, and various carotenoids. Prominent enzymatic antioxidants include catalase, glutathione peroxidase,... More; however, in this study, the researchers did not find a correlation showing lower vitamin EVitamin E is a fat-soluble vitamin that plays an important role as an antioxidant in the body. Vitamin E neutralizes harmful free radicals. Coenzyme Q10 regenerates Vitamin E after the vitamin E has neutralized free radicals. More levels linked to lower CoQ10 levels.
In this study, the levels of vitamin A and vitamin C were similar in the plasma of the patients and the controls.
Conclusion: Coenzyme Q10 and Hereditary Hemochromatosis and Iron Overload
Because the patients in this study were newly diagnosed patients (within four weeks), the researchers concluded that CoQ10 depletion occurs in unsupplemented hereditary hemochromatosis patients.
The present findings support the theory that hereditary hemochromatosis induces secondary CoQ10 deficiency [Martinefski 2021].
CoQ10 supplementation has proven to be an effective and safe adjunctive therapy in different diseases associated withCoQ10 deficiency [Garrido-Maraver 2014; Hidaka 2008].
Martinefski et al. [2021] conclude that CoQ10 supplementation may be a promising adjunctive therapeutic option for hereditary hemochromatosis to ameliorate the harmful effects of the oxidative stress associated with iron overload.
Sources
Alehagen U, Aaseth J, Johansson P. Less increase of copeptin and MR-proADM due to intervention with seleniumSelenium (symbol Se, atomic number 34) is a trace element that is an essential nutrient and an essential component of some of the most important antioxidants in the body, in particular the selenoproteins glutathione peroxidase, thioredoxin reductase, and selenoprotein P. Selenium is involved in the optimal functioning of the immune system. Professor Alehagen has pointed out that there exists a... More and coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More combined: Results from a 4-year prospective randomized double-blindA double-blind study is a study in which neither the investigators nor the study participants know which participants are receiving the active treatment and which participants are receiving the control treatment until the study has been completed and the seal on the code has been broken. More placebo-controlled trial among elderly Swedish citizens. Biofactors. 2015 Nov-Dec;41(6):443-52.
Alehagen U, Lindahl TL, Aaseth J, Svensson E, Johansson P. Levels of sP-selectin and hs-CRP Decrease with Dietary Intervention with SeleniumSelenium (symbol Se, atomic number 34) is a trace element that is an essential nutrient and an essential component of some of the most important antioxidants in the body, in particular the selenoproteins glutathione peroxidase, thioredoxin reductase, and selenoprotein P. Selenium is involved in the optimal functioning of the immune system. Professor Alehagen has pointed out that there exists a... More and Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More Combined: A Secondary Analysis of a Randomized Clinical TrialClinical trials are research studies in which various treatments, interventions, or tests are investigated for their safety and efficacy when used to detect, prevent, or treat diseases and medical conditions in patients and healthy volunteers. The gold standard for clinical trials are randomized, double-blind, placebo-controlled studies. More. PLoS One. 2015 Sep 16;10(9):e0137680.
Centers for Disease Control and Prevention. Hereditary Hemochromatosis. 2020. Retrieved from https://www.cdc.gov/genomics/disease/hemochromatosis.htm
Garrido-Maraver J, Cordero MD, Oropesa-Avila M, Vega AF, de la Mata M, Pavon AD, et al. Clinical applications of coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More. Front Biosci. 2014;19:619—33.
Hidaka T, Fujii K, Funahashi I, Fukutomi N, Hosoe K. Safety assessment of coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More (CoQ10). Biofactors. 2008;32(1-4):199—208.
Kalén A, Appelkvist EL, Dallner G. Age-related changes in the lipid compositions of rat and human tissues. Lipids. 1989 Jul;24(7):579-84.
https://pubmed.ncbi.nlm.nih.gov/2779364/
Martinefski MR, Yamasato MF, Di Carlo MB, Daruich JR, Tripodi VP. Coenzyme Q10Coenzyme Q10 molecules are fat-soluble molecules that are both synthesized in the body and ingested in the diet and in supplements. Coenzyme Q10 is synthesized in the body in the same biological pathway as cholesterol. Bio-synthesis of Coenzyme Q10 begins to decline once humans reach their adult years. The reduced production of Coenzyme Q10 cannot be compensated in any practical... More deficiency in patients with hereditary hemochromatosis. Clin Res Hepatol Gastroenterol. 2021 Mar 3;45(6):101624.
https://pubmed.ncbi.nlm.nih.gov/33676282/
The information presented in this review article is not intended as medical advice and should not be used as such.
30 October 2021
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