Tier 3 — preclinical

Biosynthesis, Deficiency, and Supplementation of Coenzyme Q

Carmine Staiano, Laura García-Corzo, David Mantle, Nadia Turton, Lauren E Millichap, Gloria Brea-Calvo, Iain Hargreaves
Antioxidants (Basel) 2023 12(7):1469

Bibliography

PubMed
PMID 37508007
PubMed Central
PMC10375973
DOI
10.3390/antiox12071469
Funding
Co-funded by the European Regional Development Fund (FEDER) and the Andalusian Regional Government under the Andalusian FEDER operational program 2014–2020, projects P18-RT-4572 and UPO-1265673.
Competing interests
David Mantle was a medical adviser to Pharma Nord (UK) Ltd. The publication states that the company had no role in study design, data collection/analysis/interpretation, manuscript writing or the decision to publish.

Study snapshot

DesignNarrative review of CoQ biosynthesis, deficiency and supplementation
ModelHuman and translational literature on CoQ biosynthetic pathways, genetic deficiency and secondary deficiency states
SampleNot applicable — review article
InterventionNo single intervention; review of endogenous biosynthesis and oral CoQ supplementation in deficiency states
DurationNot applicable
EndpointsCoQ biosynthesis pathway; Primary CoQ deficiency; Secondary CoQ deficiency; Tissue CoQ status; Clinical manifestations of deficiency; Oral supplementation challenges; Statin-associated CoQ changes

What the study showed, in plain terms

This review explains how human cells synthesize CoQ10 and why low tissue CoQ can arise through more than one mechanism. Primary CoQ deficiency results from defects in genes directly involved in the biosynthetic pathway, whereas secondary deficiency can arise from other cellular or mitochondrial dysfunctions.

The review is useful for separating true CoQ deficiency from the much looser consumer idea of having 'low CoQ10.' Tissue measurement is the more direct way to establish deficiency, and circulating concentrations do not necessarily mirror concentrations in every organ or intracellular compartment.

The authors also discuss the practical limits of supplementation: CoQ10 is highly lipophilic, absorption and tissue delivery vary, and increasing plasma concentrations does not guarantee that a deficient tissue receives an adequate amount.

Key findings

CoQ10 synthesis in human cells requires a multistep mitochondrial pathway involving numerous proteins; defects in this pathway can cause primary CoQ deficiency.

Secondary CoQ deficiency can occur without a direct mutation in a CoQ biosynthetic gene and has been described in multiple mitochondrial and systemic disorders.

Blood/plasma CoQ measurements are influenced by circulating lipoproteins and dietary/supplement exposure and should not automatically be treated as equivalent to tissue CoQ status.

Oral supplementation can increase circulating CoQ, but delivery into affected tissues—especially the central nervous system—remains a major limitation in deficiency treatment.

What this study can and cannot tell us

Narrative review rather than a prospectively registered systematic review; it synthesizes heterogeneous basic, genetic and clinical literature rather than estimating a single treatment effect.

Some supplementation discussion concerns rare genetic or mitochondrial disorders and should not be generalized to healthy adults using CoQ10 as a wellness supplement.

One author was a medical adviser to Pharma Nord, a CoQ10 manufacturer; the disclosure should remain visible even though the paper states the company had no role in the review process.

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Dr. Khaled Mahmoud, MBBChCritical Care Medicine, Longevity Science & Editorial Direction Dr. Amr Yasser, PhDCardiometabolic, Musculoskeletal, Gut & Bioavailability Dr. Abdulla Mostafa, PhDCellular Senescence, Neurological, Mitochondrial & Brain-Ageing Biology