2. Academic Validation
  3. Vitamin K2 cannot substitute Coenzyme Q10 as electron carrier in the mitochondrial respiratory chain of mammalian cells

Vitamin K2 cannot substitute Coenzyme Q10 as electron carrier in the mitochondrial respiratory chain of mammalian cells

  • Sci Rep. 2019 Apr 25;9(1):6553. doi: 10.1038/s41598-019-43014-y.
Cristina Cerqua 1 2,  Alberto Casarin 1 2,  Fabien Pierrel 3,  Luis Vazquez Fonseca 1 2,  Giampiero Viola 2 4,  Leonardo Salviati 5 6,  Eva Trevisson 7 8
Affiliations

Affiliations

  • 1 Clinical Genetics Unit, Department of Women's and Children's Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy.
  • 2 Istituto di Ricerca Pediatrica IRP Città della Speranza, Corso Stati Uniti 4, 35127, Padova, Italy.
  • 3 Univ. Grenoble Alpes, CNRS, CHU Grenoble Alpes, Grenoble INP, TIMC-IMAG, 38000, Grenoble, France.
  • 4 Pediatric Hematooncology Laboratory, Department of Women's and Children's Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy.
  • 5 Clinical Genetics Unit, Department of Women's and Children's Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy. [email protected].
  • 6 Istituto di Ricerca Pediatrica IRP Città della Speranza, Corso Stati Uniti 4, 35127, Padova, Italy. [email protected].
  • 7 Clinical Genetics Unit, Department of Women's and Children's Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy. [email protected].
  • 8 Istituto di Ricerca Pediatrica IRP Città della Speranza, Corso Stati Uniti 4, 35127, Padova, Italy. [email protected].
PMID: 31024065 DOI: 10.1038/s41598-019-43014-y
Abstract

Coenzyme Q10 (CoQ10) deficiencies are a group of heterogeneous conditions that respond to ubiquinone administration if treated soon after the onset of symptoms. However, this treatment is only partially effective due to its poor bioavailability. We tested whether vitamin K2, which was reported to act as a mitochondrial electron carrier in D. melanogaster, could mimic ubiquinone function in human CoQ10 deficient cell lines, and in yeast carrying mutations in genes required for coenzyme Q6 (CoQ6) biosynthesis. We found that vitamin K2, despite entering into mitochondria, restored neither electron flow in the respiratory chain, nor ATP synthesis. Conversely, coenzyme Q4 (CoQ4), an analog of CoQ10 with a shorter isoprenoid side chain, could efficiently substitute its function. Given its better solubility, CoQ4 could represent an alternative to CoQ10 in patients with both primary and secondary CoQ10 deficiencies.

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