Vitamin K2 is a mitochondrial electron carrier that rescues pink1 deficiency.

Vos M; Esposito G; Edirisinghe JN; Vilain S; Haddad DM; Slabbaert JR; Van Meensel S; Schaap O; De Strooper B; Meganathan R; Morais VA; Verstreken P

doi:10.1126/science.1218632

Vitamin K2 is a mitochondrial electron carrier that rescues pink1 deficiency.

PUBLISHED: 2012 Jun 08

CITE AS: Science. 2012 Jun 08; 336(6086):1306-10 https://doi.org/10.1126/science.1218632

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Human UBIAD1 localizes to mitochondria and converts vitamin K(1) to vitamin K(2). Vitamin K(2) is best known as a cofactor in blood coagulation, but in bacteria it is a membrane-bound electron carrier. Whether vitamin K(2) exerts a similar carrier function in eukaryotic cells is unknown. We identified Drosophila UBIAD1/Heix as a modifier of pink1, a gene mutated in Parkinson's disease that affects mitochondrial function. We found that vitamin K(2) was necessary and sufficient to transfer electrons in Drosophila mitochondria. Heix mutants showed severe mitochondrial defects that were rescued by vitamin K(2), and, similar to ubiquinone, vitamin K(2) transferred electrons in Drosophila mitochondria, resulting in more efficient adenosine triphosphate (ATP) production. Thus, mitochondrial dysfunction was rescued by vitamin K(2) that serves as a mitochondrial electron carrier, helping to maintain normal ATP production.

PMID: 22582012
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Relevant Sections

Neuroscience
- Neurobiology of Disease & Regeneration
Physiology
- Integrative Physiology
Genomics & Genetics
- Medical Genetics
Pharmacology & Drug Discovery
- Drug Discovery & Design
- Endocrine & Metabolic Pharmacology
- Molecular Pharmacology
- Neuropharmacology & Psychopharmacology

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