Coenzyme Q10: Role in Less Common Age-Related Disorders
Abstract
:1. Introduction
2. Multiple System Atrophy
3. Progressive Supranuclear Palsy
4. Sporadic Adult Onset Ataxia
5. Pulmonary Fibrosis
6. Late Onset Huntington’s Disease
7. Late Onset Alexander Disease
8. Late Onset Lupus
9. Late Onset Antiphospholipid Syndrome
10. Late Onset Lysosomal Storage Disorders
11. Late Onset Fibromyalgia
12. Late Onset Machado-Joseph Disease
13. Late Onset acyl-CoA Dehydrogenase Deficiency
14. Late Onset Leber’s Optic Neuropathy
15. Conclusions
Author Contributions
Funding
Conflicts of Interest
References
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Author (Ref.) | Participants | CoQ10 Status | Outcome |
---|---|---|---|
Mitsui 2016 [15] | 44 patients with MSA and 39 controls | 0.51 mg/L (MSA) vs. 0.72 mg/L (controls) | Decreased levels of plasma CoQ10 in patients with MSA regardless of the COQ2 genotype |
Kasai 2016 [16] | 18 patients with MSA, 20 patients with Parkinson’s disease, and 18 controls | Total CoQ10 level corrected by serum cholesterol significantly lower in MSA group than in Control group | Supportive evidence for the hypothesis that decreased CoQ10 levels in brain tissue result in increased MSA risk |
Du 2018 [17] | 40 MSA patients 30 patients with Parkinson’s disease and 30 healthy controls | Plasma CoQ10 levels in MSA patients lower than in controls after adjusting for age, gender, total cholesterol | Correlation between decreased CoQ10 levels and the severity of motor symptoms |
Compta 2018 [18] | 20 patients with MSA and 15 controls | Cerebrospinal fluid levels of CoQ10 lower than in controls | Support for relevance of CoQ10 in MSA |
Barca 2016 [19] | CoQ10 levels in postmortem brains of 12 MSA, 9 PD, 9 essential tremor patients, and 12 controls. | CoQ10 deficiency in MSA cerebellum associated with impaired CoQ biosynthesis and increased oxidative stress in the absence of COQ2 mutations | Evidence that CoQ10 deficiency may contribute to the pathogenesis of MSA |
Schottlaender 2016 [20] | CoQ10 levels in frozen brain tissue of 20 MSA patients and 37 elderly controls | Significant decrease (by 3–5%) in the level of CoQ10 in the cerebellum of MSA cases | Suggests that a perturbation in the CoQ10 biosynthetic pathway is associated with the pathogenesis of MSA |
Andrich 2004 [51] | Previously untreated HD patients (70.1 mg/L) had lower CoQ10 results than treated HD patients and controls | Evidence that that CoQ10 supplements may reduce impaired mitochondrial function in HD | |
Yubero 2016 [81] | Nine MPS patients | 0.32 mg/L (0.20–0.53 mg/L) in eight of the nine patients | Deficiency of CoQ10 in plasma from patients with mucopolysaccha-ridoses |
Montero 2019 [82] | 597 individuals (average age: 11 years, range one month to 43 years) | Plasma CoQ10 significantly lower in the PKU and MPS groups than in controls and neurological patients | Plasma CoQ10 monitoring recommended to prevent chronic suboptimal blood CoQ10l status |
Fu 2010 [83] | 32 Niemann-Pick disease patients | Mean serum CoQ10 level of 0.48 mg/L in Niemann-Pick patients | Reduced serum CoQ10 levels associated with oxidative stress |
Cordero 2012 [95] | 20 fibromyalgia patients and 15 controls | Decreased CoQ10, catalase, and ATP levels in BMCs from FM patients as compared to normal controls | Association of low CoQ10 levels and increased levels lipid peroxidation in BMCs from fibromyalgia patients |
Author (Ref.) | Dosage/Duration | Participants | Outcome |
---|---|---|---|
Mitsui 2017 [25] | 1200 mg/day for three years | Case study of individual in advanced stage of MSA | Substantial increase in total coenzyme Q10 levels CSF fluid and in plasma, clinical rating scales remained stable during the 3 years |
Stamelou 2008 [32] | Liquid nano-dispersion of CoQ10 (5 mg/kg/day) or matching placebo for six weeks | 21 clinically probable PSP patients (stage < or = III) | The PSP rating scale and the Frontal Assessment Battery improved slightly but significantly with the CoQ10 treatment compared to placebo, CoQ10 improved cerebral energy metabolism in PSP |
Apetauerova 2016 [33] | 2400 mg/day or placebo for up to 12 months | 61 individuals aged 40 or older who met diagnostic criteria for PSP | No significant improvement of PSP symptoms, 41% withdrawal rate |
Koroshetz 1997 [50] | 360 mg/day for 2 months. | 23 HD patients and 21 controls | CoQ10 treatment resulted in decreases in cortical lactate concentrations in 18 patients, which reversed following withdrawal of therapy |
Huntington Study Group 2001 [54] | 300 mg twice daily for 30 months | 347 HD patients | No significant slowing of functional decline in early HD |
McGarry 2017 [55] | 2400 mg/day or matching placebo for 60 months | 609 HD patients | No significant difference in patients’ Total Functional Capacity score |
Perez-Sanchez 2017 [74] | 200 mg/day for one month | 36 patients with antiphospholipid syndrome | Improved endothelial function, reduced expression of pro-thrombotic and pro-inflammatory mediators |
Fu 2010 [83] | Information on the dosage of CoQ10 or the duration of the study not provided in the manuscript | 9 Niemann-Pick disease patients | Supplementation increased serum status to 1.09 mg/L but did not significantly raise the reduced CoQ10 fraction |
Cordero 2013 [92] | 300 mg/day or placebo for 40 days | 20 fibromyalgia patients | Supplementation reduced pain and fatigue by more than 50% |
Alcocer-Gomez 2014 [93] | 300 mg/day or placebo for 40 days | 20 fibromyalgia patients | Improvement in depression symptoms |
Alcocer-Gomez 2017 [94] | 300 mg/day or placebo for 40 days | 20 fibromyalgia patients | Improvement in the clinical symptoms determined by FIQ questionnaire and PSQI index |
Cordero 2012 [95] | 300 mg/day for three months | 20 fibromyalgia patients and 15 controls | Improvement in clinical symptoms and headache in fibromyalgia, increase in intracellular ATP levels in BMCs |
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Mantle, D.; Hargreaves, I.P. Coenzyme Q10: Role in Less Common Age-Related Disorders. Antioxidants 2022, 11, 2293. https://doi.org/10.3390/antiox11112293
Mantle D, Hargreaves IP. Coenzyme Q10: Role in Less Common Age-Related Disorders. Antioxidants. 2022; 11(11):2293. https://doi.org/10.3390/antiox11112293
Chicago/Turabian StyleMantle, David, and Iain P. Hargreaves. 2022. "Coenzyme Q10: Role in Less Common Age-Related Disorders" Antioxidants 11, no. 11: 2293. https://doi.org/10.3390/antiox11112293