Coenzyme Q10 in the Treatment of Mitochondrial Disease

  title={Coenzyme Q10 in the Treatment of Mitochondrial Disease},
  author={Viruna Neergheen and Annapurna Chalasani and Luke Wainwright and D{\`e}lia Yubero and Raquel Montero and Rafael Artuch and Iain P. Hargreaves},
Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10 (CoQ10) and related synthetic quinones are the only agents to date that have pr... 

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Coenzyme Q10 and the exclusive club of diseases that show a limited response to treatment
Evidence of mitochondrial dysfunction in MS, PD and mitochondrial ETC disorders is identified and the inability of Co10 supplementation to elicit significant clinical outcome in these disorders and possible flaws in these studies will be discussed.
The Roles of Coenzyme Q in Disease: Direct and Indirect Involvement in Cellular Functions
The complete knowledge of the various cellular CoQ functions is essential to provide a rational basis for its possible therapeutic use, not only in diseases characterized by primary CoQ deficiency, but also in large number of diseases in which its secondary deficiency has been found.
Current biochemical treatments of mitochondrial respiratory chain disorders
The inclusion of pharmacotherapies that target MRC function, cellular antioxidant status and mitochondrial biogenesis in the treatment regime of patients may be appropriate to ameliorate the defects in these parameters that contribute to disease pathophysiology.
The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification
Insight is provided into the association between lysosomal pH and cellular CoQ10 status and the possibility that a deficit in the status of this isoprenoid may result in an impairment of lysOSomal acidification.
Therapeutic Approaches to Treat Mitochondrial Diseases: “One-Size-Fits-All” and “Precision Medicine” Strategies
The state of the art on experimental treatments for mitochondrial diseases is reviewed, presenting “one-size-fits-all” approaches and precision medicine strategies.
Bioactivity Profiles of Cytoprotective Short-Chain Quinones
An unexpected mode of action for SCQs appears to involve a modification of NQO1-dependent signaling rather than a protective effect by the reduced quinone itself, which presents a new selection strategy to identify and develop the most promising compounds towards their clinical use.
Coenzyme Q10, Ageing and the Nervous System: An Overview
The potential therapeutic role in these age-related neurological disorders of supplementary coenzyme Q10, a vitamin-like substance of vital importance for normal mitochondrial function and as an antioxidant, is reviewed.
CoQ10 and Aging
There is currently not enough evidence to recommend CoQ10 supplementation as an anti-aging anti-oxidant therapy, despite the current lay public interest in supplementing with CoQ 10.
No Effect of Coenzyme Q10 on Cognitive Function, Psychological Symptoms, and Health-related Outcomes in Schizophrenia and Schizoaffective Disorder
The results of the study suggest that CoQ10 supplementation at 300 mg/day for 6 months is unlikely to be beneficial for cognitive, psychological and health-related outcomes in schizophrenia and schizoaffective disorder.


Coenzyme Q10 as a therapy for mitochondrial disease.
  • I. Hargreaves
  • Biology
    The international journal of biochemistry & cell biology
  • 2014
Early coenzyme Q10 supplementation in primary coenzyme Q10 deficiency.
The results of long-term coen enzyme Q10 supplementation in two patients with coenzyme Q10 deficiency caused by a homozygous missense mutation in the COQ2 gene are described.
Clinical improvement after administration of coenzyme Q10 in a patient with mitochondrial encephalomyopathy
High doses of coenzyme Q10 (CoQ) were administered in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes and clinical improvement with decreased serum lactate and pyruvate levels was observed.
Treatment of Kearns‐Sayre syndrome with coenzyme Q10
Although the mitochondrial fraction was increased in muscles from KSS patients, CoQ content was slightly low, and Administration of 120 to 150 mg/d of CoQ improved abnormal metabolism of pyruvate and NADH oxidation in skeletal muscle.
Coenzyme Q10 with multiple vitamins is generally ineffective in treatment of mitochondrial disease
It is suggested that coenzyme Q10 plus vitamin therapy does not significantly improve mitochondrial oxidative metabolism in patients with mitochondrial disease in general and any clinical benefit that may follow from short-term administration appears slight.
Biochemical Diagnosis of Coenzyme Q10 Deficiency
In this article, the biochemical methods used in the diagnosis of human CoQ10 deficiency are reviewed and the most appropriate tissues for this evaluation are indicated.
Genetics of Coenzyme Q10 Deficiency
It is of critical importance that physicians promptly recognize these disorders because most patients respond to oral administration of CoQ10, a clinically and genetically heterogeneous disorder.
Genetic bases and clinical manifestations of coenzyme Q10 (CoQ10) deficiency
Patients with both primary and secondary deficiencies benefit from high-dose oral supplementation with CoQ10, and treatment can stop the progression of both SRNS and encephalopathy, hence the critical importance of a prompt diagnosis.