Depletion and Supplementation of Coenzyme Q10 in Secondary Deficiency Disorders

Mantle, David; Turton, Nadia; Hargreaves, Iain P.

doi:10.31083/j.fbl2712322

Cited by 5 publications

(2 citation statements)

References 100 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…At least 10 genes are required for the biosynthesis of functional CoQ10, a mutation in any one of which can result in a deficit in CoQ10 status [ 3 ]. Many of the data relating to CoQ10 biosynthesis were initially obtained from studies in yeast, with deficiencies corresponding to the above genes denoted as CoQ1 to CoQ11 (numbering refers to date order of identification) [ 9 ].…”

Section: Biosynthesis Of Coq10mentioning

confidence: 99%

“…Secondary CoQ10 disorders are relatively common and may occur for a variety of reasons; these include mutations in genes not directly related to the synthetic pathway, the oxidative-stress-induced reduction of CoQ10, and the effects of pharmacological agents such as statins. The role of CoQ10 deficiency and the benefits of supplementation in secondary CoQ10 disorders have been reviewed recently [ 3 ] and are not further considered in the present article.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Primary Coenzyme Q10 Deficiency: An Update

Mantle,

Millichap,

Castro-Marrero

et al. 2023

Antioxidants

Self Cite

View full text Add to dashboard Cite

Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and extra-mitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation, CoQ10 serves as a lipid soluble antioxidant and plays an important role in fatty acid beta-oxidation and pyrimidine and lysosomal metabolism, as well as directly mediating the expression of a number of genes, including those involved in inflammation. Due to the multiplicity of roles in cell function, it is not surprising that a deficiency in CoQ10 has been implicated in the pathogenesis of a wide range of disorders. CoQ10 deficiency is broadly divided into primary and secondary types. Primary CoQ10 deficiency results from mutations in genes involved in the CoQ10 biosynthetic pathway. In man, at least 10 genes are required for the biosynthesis of functional CoQ10, a mutation in any one of which can result in a deficit in CoQ10 status. Patients may respond well to oral CoQ10 supplementation, although the condition must be recognised sufficiently early, before irreversible tissue damage has occurred. In this article, we have reviewed clinical studies (up to March 2023) relating to the identification of these deficiencies, and the therapeutic outcomes of CoQ10 supplementation; we have attempted to resolve the disparities between previous review articles regarding the usefulness or otherwise of CoQ10 supplementation in these disorders. In addition, we have highlighted several of the potential problems relating to CoQ10 supplementation in primary CoQ10 deficiency, as well as identifying unresolved issues relating to these disorders that require further research.

show abstract

Section: Biosynthesis Of Coq10mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Primary Coenzyme Q10 Deficiency: An Update

Mantle,

Millichap,

Castro-Marrero

et al. 2023

Antioxidants

Self Cite

View full text Add to dashboard Cite

show abstract

Multiple sclerosis: is there a role for coenzyme Q10?

Mantle

2023

British Journal of Neuroscience Nursing

View full text Add to dashboard Cite

Mitochondrial dysfunction, oxidative stress and inflammation have been implicated in the pathogenesis of multiple sclerosis (MS). Therefore, there is a rationale for the use of supplementary coenzyme Q10 (CoQ10) as a potential novel therapeutic agent for this disorder, based on the key roles of this vitamin-like substance in normal mitochondrial function, as an antioxidant and as an anti-inflammatory agent. In this article, the pre-clinical and clinical studies relating to the use of supplementary CoQ10 for the treatment of MS is reviewed.

show abstract

New Insights on the Uptake and Trafficking of Coenzyme Q

Guile,

Jain,

Anderson

et al. 2023

Antioxidants

View full text Add to dashboard Cite

Coenzyme Q (CoQ) is an essential lipid with many cellular functions, such as electron transport for cellular respiration, antioxidant protection, redox homeostasis, and ferroptosis suppression. Deficiencies in CoQ due to aging, genetic disease, or medication can be ameliorated by high-dose supplementation. As such, an understanding of the uptake and transport of CoQ may inform methods of clinical use and identify how to better treat deficiency. Here, we review what is known about the cellular uptake and intracellular distribution of CoQ from yeast, mammalian cell culture, and rodent models, as well as its absorption at the organism level. We discuss the use of these model organisms to probe the mechanisms of uptake and distribution. The literature indicates that CoQ uptake and distribution are multifaceted processes likely to have redundancies in its transport, utilizing the endomembrane system and newly identified proteins that function as lipid transporters. Impairment of the trafficking of either endogenous or exogenous CoQ exerts profound effects on metabolism and stress response. This review also highlights significant gaps in our knowledge of how CoQ is distributed within the cell and suggests future directions of research to better understand this process.

show abstract

Depletion and Supplementation of Coenzyme Q10 in Secondary Deficiency Disorders

Cited by 5 publications

References 100 publications

Primary Coenzyme Q10 Deficiency: An Update

Primary Coenzyme Q10 Deficiency: An Update

Multiple sclerosis: is there a role for coenzyme Q10?

New Insights on the Uptake and Trafficking of Coenzyme Q

Contact Info

Product

Resources

About