7 Signs & Symptoms of Coenzyme Q10 Deficiency & Treatment – Research Studies

What is Coenzyme Q10?

Coenzyme Q10 (CoQ10), also known as ubiquinone, is present in the cell membrane of every cell in the human body. CoQ10 is a vitamin-like molecule that is fat-soluble in nature. Not only do humans endogenously synthesize CoQ10, but they also consume the molecule in their diet. CoQ10 or ubiquinone is integral to the transfer of electrons and the production of adenosine triphosphate (ATP) in the oxidative respiratory chain of mitochondria. This molecule also promotes the production of antioxidants and reduction in the pro-oxidative compounds in the body. CoQ10 preserves nitric oxide, thus, protecting the blood vessels and enhancing blood flow. [1]  

CoQ10 supplements are well-tolerated by individuals since this molecule is naturally present in the human body, however, higher doses of these supplements may cause mild insomnia. While CoQ10 supplements are recommended by healthcare providers and available over the counter, these are not approved by the Food and Drug Administration (FDA). [1] 

Why Coenzyme Q10 is Important?

The importance of CoQ10 in the normal physiology of the human body is represented by the development of medical conditions associated with low levels of CoQ10 in the body. These include cancer, neurodegenerative conditions, migraine, diabetes, cardiovascular disorders, mitochondrial diseases, fibromyalgia, and muscular diseases. In patients with congestive heart failure, CoQ10 supplementation improves contractility of the left ventricle, functional capacity, and endothelial function. In patients suffering from fibromyalgia, CoQ10 supplementation reduces morning tiredness, pain, and fatigue. [1] 

This safe non-FDA-approved dietary supplement has several complementary and alternative medicine applications. In order to potentiate the benefits and counter the adverse outcomes of CoQ10 supplementation, healthcare providers shall be vigilant while prescribing the supplements, particularly in liver or kidney disease patients. [1] 

7 Signs & Symptoms of Coenzyme Q10 Deficiency

1.Seizures 

Research has demonstrated that an insufficient quantity of CoQ10 in the body is associated with the frequency and duration of seizures in patients suffering from epilepsy. CoQ10 deficiency is implicated in the pathogenesis of seizures, however, there is no relationship between the levels of CoQ10 and the type of seizure. The possible mechanism is based on the diminished production of endogenous antioxidants and increased production of mitochondrial superoxide anion, which damages different components in the cells. [2] 

2. Hearing Loss

Primary deficiency of CoQ10 can lead to sensorineural hearing loss. CoQ10 monooxygenase 6 or COQ6 is an enzyme that is important for the biosynthesis of CoQ10. COQ6 has high expression in the spinal ligament and stria vascularis of the inner ear. The importance of CoQ10 in hearing loss is further supported by the notion that CoQ10 replacement therapy improves hearing loss. [3]

3. Kidney Dysfunction 

Primary deficiency of CoQ10 leads to the development of nephrotic syndrome in young adults and children. Renal deficiency of this enzyme causes respiratory chain deficiency, which promotes oxidative stress. This may lead to damage to the renal structures and give rise to proteinuria. Supplementation with CoQ10 is known to stabilize proteinuria and also significantly increases the protein/creatinine ratio. CoQ10 supplementation is also implicated in the preservation of kidney structure. [4]

4. Intellectual Disability 

Several genes encode various enzymes that are implicated in the biosynthesis of CoQ10. The deficiency of COQ5, the gene that encodes C-methyltransferase, which performs C-methylation during the synthesis of CoQ10, is associated with mild non-progressive atrophy of the cerebellum on brain magnetic resonance imaging (MRI). The clinical findings of CoQ10 deficiency in such cases include intellectual disability, cerebellar ataxia, and epilepsy. [5]

5. Muscle Weakness 

The deficiency of CoQ10 causes impaired oxidative phosphorylation, which leads to decreased production of ATP. This culminates in impaired metabolism of muscle energy. This mechanism is also associated with statin-related myopathy. Supplementation of CoQ10 aids in the reduction of muscular symptoms, preventing their interference with daily activities. [6]

6. Vision Loss 

CoQ10 is detected in the retina and its levels decline with aging, corresponding to the increase in oxidative stress. Oxidative stress contributes to the retinal degeneration process. Research suggests that supplementation with CoQ10 inhibits the synthesis of reactive oxygen species and protects the cells in the retina from oxidative damage. This helps with the treatment of glaucoma and other age-related macular disorders. [7]

7. Cardiac Symptoms

A deficiency of CoQ10 in the cardiac tissue can give rise to multiple symptoms. These may correspond to heart failure and hypertrophic cardiomyopathy. The depletion of CoQ10 status in cardiovascular status is related to weakened cellular antioxidant status along with the impaired function of mitochondria and energy supply of the affected cells. Supplementation with CoQ10 has significant implications for improvement in cardiac-related mortality. Significant symptomatic improvement is also observed in heart failure patients with CoQ10 supplementation. [8]

Coenzyme Q10 Deficiency 治療

The treatment of CoQ10 deficiency in the body can be countered using the intake of CoQ10 dietary supplements. It shall be noted that toxicity of this supplement does not exist even at the highest dose of oral supplementation. CoQ10 dietary supplements are contraindicated in patients with liver or kidney disease patients taking chemotherapeutic drugs, and diabetes patients. Since this molecule has chemical similarity with vitamin K, it may potential interaction with warfarin, causing failure of warfarin therapy. [1]

Take advantage of free consultation with one of our Health Coach through the chat icon on the website to determine of CoQ10 deficiency along with the identification of appropriate dosages. ASTR CoQ10 Active Supplements are recommended for patients who suffer from a deficiency of this molecule in the body. 

參考 

1. Sood B, Keenaghan M. Coenzyme Q10. [Updated 2022 Jan 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK531491/
2. Simani, L., Rezaei, O., Ryan, F., Sadeghi, M., Hooshmandi, E., Ramezani, M., & Pakdaman, H. (2020). Coenzyme Q10 Insufficiency Contributes to the Duration and Frequency of Seizures in Epileptic Patients. Basic and clinical neuroscience, 11(6), 765–771. https://doi.org/10.32598/bcn.11.6.1100.1  
3. Nam, D. W., Park, S. S., Lee, S. M., Suh, M. W., Park, M. K., Song, J. J., Choi, B. Y., Lee, J. H., Oh, S. H., Moon, K. C., Ahn, Y. H., Kang, H. G., Cheong, H. I., Kim, J. H., & Lee, S. Y. (2022). Effects of CoQ10 Replacement Therapy on the Audiological Characteristics of Pediatric Patients with COQ6 Variants. BioMed research international, 2022, 5250254. https://doi.org/10.1155/2022/5250254
4. Kleiner, G., Barca, E., Ziosi, M., Emmanuele, V., Xu, Y., Hidalgo-Gutierrez, A., Qiao, C., Tadesse, S., Area-Gomez, E., Lopez, L. C., & Quinzii, C. M. (2018). CoQ₁₀ supplementation rescues nephrotic syndrome through normalization of H₂S oxidation pathway. Biochimica et biophysica acta. Molecular basis of disease, 1864(11), 3708–3722. https://doi.org/10.1016/j.bbadis.2018.09.002
5. Münch J, Prasuhn J, Laugwitz L, Fung C-W, Chung BH-Y, Bellusci M, Mayatepek E, Klee D, Distelmaier F. Neuroimaging in Primary Coenzyme-Q₁₀-Deficiency Disorders. Antioxidants. 2023; 12(3):718. https://doi.org/10.3390/antiox12030718
6. Skarlovnik, A., Janić, M., Lunder, M., Turk, M., & Šabovič, M. (2014). Coenzyme Q10 supplementation decreases statin-related mild-to-moderate muscle symptoms: a randomized clinical study. Medical science monitor : international medical journal of experimental and clinical research, 20, 2183–2188. https://doi.org/10.12659/MSM.890777
7. Zhang, X., Tohari, A. M., Marcheggiani, F., Zhou, X., Reilly, J., Tiano, L., & Shu, X. (2017). Therapeutic Potential of Co-enzyme Q10 in Retinal Diseases. Current medicinal chemistry, 24(39), 4329–4339. https://doi.org/10.2174/0929867324666170801100516
8. Hargreaves, I., Heaton, R. A., & Mantle, D. (2020). Disorders of Human Coenzyme Q10 Metabolism: An Overview. International journal of molecular sciences, 21(18), 6695. https://doi.org/10.3390/ijms21186695