Vitamin K 2 Is a Mitochondrial Electron Carrier That Rescues Pink1 Deficiency

• DOI: 10.1126/science.1218632
• Publication Date: 2012-05-11T04:46:28Z
• Authors: Melissa Vos; Giovanni Esposito; Janaka N. Edirisi...; Sven Vilain; Dominik M. Haddad; Jan R. Slabbaert; Stefanie Van Meensel; Onno Schaap; Bart De Strooper; R. Meganathan; Vanessa A. Morais; Patrik Verstreken
• Keywords: Membrane Potential, Mitochondrial; 0301 basic medicine; Ubiquinone; Ubiquitin-Protein Ligases; Genes, Insect; Vitamin K 2; Protein Serine-Threonine Kinases; Protein-Serine-Threonine Kinases; Mitochondria; Mitochondria, Muscle; Electron Transport; 03 medical and health sciences; Adenosine Triphosphate; Oxygen Consumption; Flight, Animal; Mutation; Escherichia coli; Animals; Drosophila Proteins; Drosophila

Keeping Mitochondria in the Pink

Pink1 is a mitochondrial kinase, and loss of Pink1 function in flies and mice results in the accumulation of inefficient mitochondria. In a screen for modifiers of the Parkinson-associated gene, pink1 ,

Vos et al.

(p. 1306 , published online 10 May; see the Perspective by

Bhalerao and Clandinin

) identified the fruit fly homolog of UBIAD1, “Heix.” UBIAD1 was localized in mitochondria and was able to convert vitamin K 1 into vitamin K 2 /menaquinone (MK- n, n the number of prenylgroups). In bacteria, vitamin K 2 /MK- n acts as an electron carrier in the membrane and, similarly, in Drosophila , mitochondrial vitamin K 2 appeared to act as an electron carrier to facilitate adenosine triphosphate production. Fruit flies that lack heix showed severe mitochondrial defects that could be rescued by administering vitamin K 2 .