Secondary coenzyme Q deficiency in neurological disorders

Guéguen, Naïg; Baris, Olivier; Lenaers, Guy; Reynier, Pascal; Spinazzi, Marco

doi:10.1016/j.freeradbiomed.2021.01.017

Cited by 11 publications

(12 citation statements)

References 186 publications

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“…In fact, while it is generally assumed that CoQ10 is absorbed by passive diffusion and membrane recycling, differences in endogenous tissue and subcellular content as well as absorption rate are reported [ 51 ]. At the subcellular level, the highest CoQ content is found in mitochondria and the tissues most rich in CoQ are cardiac, skeletal muscle, and liver, where mitochondria are abundant [ 52 ]. Notably despite the large amounts and the critical role of the cofactor in these tissues, they appear particularly refractory to exogenous CoQ uptake compared to other tissues such as blood, spleen and liver [ 51 ].…”

Section: Discussionmentioning

confidence: 99%

Coenzyme Q10 Supplementation in Statin Treated Patients: A Double-Blinded Randomized Placebo-Controlled Trial

et al. 2022

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Myalgia and new-onset of type 2 diabetes have been associated with statin treatment, which both could be linked to reduces coenzyme Q10 (CoQ10) in skeletal muscle and impaired mitochondrial function. Supplementation with CoQ10 focusing on levels of CoQ10 in skeletal muscle and mitochondrial function has not been investigated in patients treated with statins. To investigate whether concomitant administration of CoQ10 with statins increases the muscle CoQ10 levels and improves the mitochondrial function, and if changes in muscle CoQ10 levels correlate with changes in the intensity of myalgia. 37 men and women in simvastatin therapy with and without myalgia were randomized to receive 400 mg CoQ10 daily or matched placebo tablets for eight weeks. Muscle CoQ10 levels, mitochondrial respiratory capacity, mitochondrial content (using citrate synthase activity as a biomarker), and production of reactive oxygen species were measured before and after CoQ10 supplementation, and intensity of myalgia was determined using the 10 cm visual analogue scale. Muscle CoQ10 content and mitochondrial function were unaltered by CoQ10 supplementation. Individual changes in muscle CoQ10 levels were not correlated with changes in intensity of myalgia. CoQ10 supplementation had no effect on muscle CoQ10 levels or mitochondrial function and did not affect symptoms of myalgia.

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Section: Discussionmentioning

confidence: 99%

Coenzyme Q10 Supplementation in Statin Treated Patients: A Double-Blinded Randomized Placebo-Controlled Trial

et al. 2022

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“…Although these processes are the main features of CoQ deficiency, other processes where CoQ is involved contribute significantly to the development of the diseases [ 57 ]. Generally, CoQ deficiency has been associated with many age-related diseases [ 39 ], neurological disorders [ 93 ], kidney and liver diseases [ 94 , 95 ], and heart failure [ 96 ], among others.…”

Section: Coq Deficiency Syndromementioning

confidence: 99%

Coenzyme Q at the Hinge of Health and Metabolic Diseases

et al. 2021

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Coenzyme Q is a unique lipidic molecule highly conserved in evolution and essential to maintaining aerobic metabolism. It is endogenously synthesized in all cells by a very complex pathway involving a group of nuclear genes that share high homology among species. This pathway is tightly regulated at transcription and translation, but also by environment and energy requirements. Here, we review how coenzyme Q reacts within mitochondria to promote ATP synthesis and also integrates a plethora of metabolic pathways and regulates mitochondrial oxidative stress. Coenzyme Q is also located in all cellular membranes and plasma lipoproteins in which it exerts antioxidant function, and its reaction with different extramitochondrial oxidoreductases contributes to regulate the cellular redox homeostasis and cytosolic oxidative stress, providing a key factor in controlling various apoptosis mechanisms. Coenzyme Q levels can be decreased in humans by defects in the biosynthesis pathway or by mitochondrial or cytosolic dysfunctions, leading to a highly heterogeneous group of mitochondrial diseases included in the coenzyme Q deficiency syndrome. We also review the importance of coenzyme Q levels and its reactions involved in aging and age-associated metabolic disorders, and how the strategy of its supplementation has had benefits for combating these diseases and for physical performance in aging.

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“…It some cases the symptoms predominantly affect a particular organ or tissue (e.g., kidney-or cerebellum-limited phenotypes) [8,[14][15][16]. Secondary CoQ10 deficiency refers to all the conditions in which the etiology of a CoQ10 deficiency is not a molecular lesion in the CoQ10 biosynthetic pathway [17,18]. In fact, a variety of conditions have been found to be associated with CoQ10 deficiency.…”

Section: Introductionmentioning

confidence: 99%

The efficacy of coenzyme Q₁₀treatment in alleviating the symptoms of primary coenzyme Q₁₀deficiency: a systematic review

Wang

Hekimi

2022

Preprint

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Coenzyme Q10 (CoQ10) is necessary for mitochondrial electron transport. Mutations in CoQ10 biosynthetic genes cause primary CoQ10 deficiency (PCoQD) and manifest as mitochondrial disorders. It is often stated that PCoQD patients can be treated by oral CoQ10 supplementation. To test this, we compiled all studies describing PCoQD patients up to May 2022. We excluded studies with no data on CoQ10 treatment, or with insufficient description of effectiveness. Out of 303 PCoQD patients identified, we retained 89 cases, of which 24 reported improvements after CoQ10 treatment (27.0%). In five cases, the patient’s condition was reported to deteriorate after halting of CoQ10 treatment. 12 cases reported improvement in the severity of ataxia, and 5 cases in the severity of proteinuria. Only a subjective description of improvement was reported for four patients described as responding. All reported responses were partial improvements of only some symptoms. For PCoQD patients, CoQ10 supplementation is replacement therapy. Yet, there is only very weak evidence for the efficacy of the treatment. Our findings thus suggest a need for caution when seeking to justify the widespread use of CoQ10 for the treatment of any disease or as dietary supplement.HighlightsOnly 27% of primary CoQ10 deficiency patients benefited from CoQ10 supplementation.Studies of the effects of supplementation necessarily lacked controls and blinding.All reported positive responses to treatment only partially improved few symptoms.CoQ10 supplementation for the treatment of any disease should be questioned.Firm evidence of benefits requires randomize, controlled trials of CoQ10 therapy.Graphic Abstract

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Secondary coenzyme Q deficiency in neurological disorders

Cited by 11 publications

References 186 publications

Coenzyme Q10 Supplementation in Statin Treated Patients: A Double-Blinded Randomized Placebo-Controlled Trial

Coenzyme Q10 Supplementation in Statin Treated Patients: A Double-Blinded Randomized Placebo-Controlled Trial

Coenzyme Q at the Hinge of Health and Metabolic Diseases

The efficacy of coenzyme Q₁₀treatment in alleviating the symptoms of primary coenzyme Q₁₀deficiency: a systematic review

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Secondary coenzyme Q deficiency in neurological disorders

Cited by 11 publications

References 186 publications

Coenzyme Q10 Supplementation in Statin Treated Patients: A Double-Blinded Randomized Placebo-Controlled Trial

Coenzyme Q10 Supplementation in Statin Treated Patients: A Double-Blinded Randomized Placebo-Controlled Trial

Coenzyme Q at the Hinge of Health and Metabolic Diseases

The efficacy of coenzyme Q10treatment in alleviating the symptoms of primary coenzyme Q10deficiency: a systematic review

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The efficacy of coenzyme Q₁₀treatment in alleviating the symptoms of primary coenzyme Q₁₀deficiency: a systematic review