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

Abstract

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 .