Vitamin K2 is a mitochondrial electron carrier that rescues pink1 deficiency

Melissa Vos, Giovanni Esposito, Janaka N. Edirisinghe, Sven Vilain, Dominik M. Haddad, Jan R. Slabbaert, Stefanie Van Meensel, Onno Schaap, Bart De Strooper, R. Meganathan, Vanessa A. Morais, Patrik Verstreken*

*Corresponding author for this work
217 Citations (Scopus)


Human UBIAD1 localizes to mitochondria and converts vitamin K1 to vitamin K2. Vitamin K2 is best known as a cofactor in blood coagulation, but in bacteria it is a membrane-bound electron carrier. Whether vitamin K2 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 K2 was necessary and sufficient to transfer electrons in Drosophila mitochondria. Heix mutants showed severe mitochondrial defects that were rescued by vitamin K2, and, similar to ubiquinone, vitamin K2 transferred electrons in Drosophila mitochondria, resulting in more efficient adenosine triphosphate (ATP) production. Thus, mitochondrial dysfunction was rescued by vitamin K2 that serves as a mitochondrial electron carrier, helping to maintain normal ATP production.

Original languageEnglish
Issue number6086
Pages (from-to)1306-1310
Number of pages5
Publication statusPublished - 08.06.2012


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