Evidence map›Paper›PMID 32975579›Full record

ArticleHuman molecular genetics2020

Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism.

Pilar González-García, Agustín Hidalgo-Gutiérrez, Cristina Mascaraque, Eliana Barriocanal-Casado, Mohammed Bakkali, Marcello Ziosi, Ussipbek Botagoz Abdihankyzy, Sabina Sánchez-Hernández, Germaine Escames, Holger Prokisch and 3 more

Open access · hybridAbstract read
In one paragraph

Article in Human molecular genetics, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.

0numbers the graph read from it
0cells of the map it votes in
23citing papers in PubMed
1.3field-weighted citation impact, top 20% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

23 citing papers in PubMed, 31 citations in OpenAlex.

  1. Review
  2. Bypass Treatments for Primary Coenzyme Q10 Deficiency: An Update.International journal of molecular sciences · 2026
    Review
  3. Article
  4. Review
  5. Review
  6. Article
  7. Article
  8. Coenzyme QFree radical biology & medicine · 2025
    Article
  9. SQOR as a metabolic rheostat of HFrontiers in cell and developmental biology · 2025
    Review
  10. Article
  11. Article
  12. Understanding coenzyme Q.Physiological reviews · 2024
    Review
  13. Article
  14. New Insights on the Uptake and Trafficking of Coenzyme Q.Antioxidants (Basel, Switzerland) · 2023
    Review
  15. Advances of HAntioxidants (Basel, Switzerland) · 2023
    Review
  16. Total and reduced/oxidized forms of coenzyme QMolecular genetics and metabolism reports · 2023
    Article
  17. Review
  18. CoQNutrients · 2022
    Review
  19. Article
  20. Coenzyme Q at the Hinge of Health and Metabolic Diseases.Antioxidants (Basel, Switzerland) · 2021
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

13 authors at 5 institutions in 4 countries.

Pilar González-GarcíaInstituto de Biotecnología, Centro de Investigación Biomédica, Universidad de Granada, Granada 18016, Spain.
Agustín Hidalgo-GutiérrezInstituto de Biotecnología, Centro de Investigación Biomédica, Universidad de Granada, Granada 18016, Spain.
Cristina MascaraqueDepartamento de Fisiología, Facultad de Medicina, Universidad de Granada, Granada 18016, Spain.
Eliana Barriocanal-CasadoInstituto de Biotecnología, Centro de Investigación Biomédica, Universidad de Granada, Granada 18016, Spain.
Mohammed BakkaliDepartamento de Genética, Facultad de Ciencias, Universidad de Granada, Granada 18071, Spain.
Marcello ZiosiDepartment of Neurology, Columbia University Medical Center, New York 10032, NY, USA.
Ussipbek Botagoz AbdihankyzyDepartment of Biophysics and Biomedicine, Al-Farabi Kazakh National University, Almaty 050040, Kazakhstan.
Sabina Sánchez-HernándezGenomic Medicine Department, Centre for Genomics and Oncological Research, Granada 18007, Spain.
Germaine EscamesInstituto de Biotecnología, Centro de Investigación Biomédica, Universidad de Granada, Granada 18016, Spain.
Holger ProkischInstitute of Human Genetics, Technische Universität München, München 81675, Germany.
Francisco MartínGenomic Medicine Department, Centre for Genomics and Oncological Research, Granada 18007, Spain.
Catarina M QuinziiDepartment of Neurology, Columbia University Medical Center, New York 10032, NY, USA.
Luis C LópezInstituto de Biotecnología, Centro de Investigación Biomédica, Universidad de Granada, Granada 18016, Spain.
Universidad de Granada · ESColumbia University Irving Medical Center · USPfizer-University of Granada-Junta de Andalucía Centre for Genomics and Oncological Research · ESAl-Farabi Kazakh National University · KZTechnical University of Munich · DE

Funding

Tech-CoreP01HD080642 · NICHD · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI QUINZII, CATARINA M. · 2014 to 2018
$7.8M
NICHD NIH HHS P01 HD080642
6 · The paper itself

Abstract

Abnormalities of one carbon, glutathione and sulfide metabolisms have recently emerged as novel pathomechanisms in diseases with mitochondrial dysfunction. However, the mechanisms underlying these abnormalities are not clear. Also, we recently showed that sulfide oxidation is impaired in Coenzyme Q10 (CoQ10) deficiency. This finding leads us to hypothesize that the therapeutic effects of CoQ10, frequently administered to patients with primary or secondary mitochondrial dysfunction, might be due to its function as cofactor for sulfide:quinone oxidoreductase (SQOR), the first enzyme in the sulfide oxidation pathway. Here, using biased and unbiased approaches, we show that supraphysiological levels of CoQ10 induces an increase in the expression of SQOR in skin fibroblasts from control subjects and patients with mutations in Complex I subunits genes or CoQ biosynthetic genes. This increase of SQOR induces the downregulation of the cystathionine β-synthase and cystathionine γ-lyase, two enzymes of the transsulfuration pathway, the subsequent downregulation of serine biosynthesis and the adaptation of other sulfide linked pathways, such as folate cycle, nucleotides metabolism and glutathione system. These metabolic changes are independent of the presence of sulfur aminoacids, are confirmed in mouse models, and are recapitulated by overexpression of SQOR, further proving that the metabolic effects of CoQ10 supplementation are mediated by the overexpression of SQOR. Our results contribute to a better understanding of how sulfide metabolism is integrated in one carbon metabolism and may explain some of the benefits of CoQ10 supplementation observed in mitochondrial diseases.

Indexed as

AnimalsAtaxiaCarbonElectron TransportElectron Transport Complex IFibroblastsGlutathioneHumansMiceMice, Inbred C57BLMitochondriaMitochondrial DiseasesMuscle WeaknessOxidoreductases Acting on Sulfur Group DonorsSkinSulfidesCarboncoenzyme Q10Electron Transport Complex IGlutathioneOxidoreductases Acting on Sulfur Group DonorsSQOR protein, humanSulfidesUbiquinoneVitamins

Identifiers

PMID32975579
PMCPMC7724311
OpenAlexW3088625186

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.