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Vitamin K₂ Is a Mitochondrial Electron Carrier That Rescues Pink1 Deficiency
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Vitamin K₂ Is a Mitochondrial Electron Carrier That Rescues Pink1 Deficiency
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Journal Article
Vitamin K₂ Is a Mitochondrial Electron Carrier That Rescues Pink1 Deficiency
2012
Overview
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.
Publisher
American Association for the Advancement of Science,The American Association for the Advancement of Science
Subject
/ Adenosine Triphosphate - metabolism
/ Animals
/ ATP
/ bacteria
/ Biological and medical sciences
/ Cell structures and functions
/ Drosophila Proteins - deficiency
/ Drosophila Proteins - genetics
/ Drosophila Proteins - metabolism
/ Escherichia coli - metabolism
/ Fruits
/ Fundamental and applied biological sciences. Psychology
/ genes
/ Larvae
/ Membrane Potential, Mitochondrial
/ mice
/ Mitochondria - ultrastructure
/ Mitochondria and cell respiration
/ Mitochondria, Muscle - metabolism
/ Mitochondria, Muscle - ultrastructure
/ Molecular and cellular biology
/ Mutation
/ Protein-Serine-Threonine Kinases - deficiency
/ Protein-Serine-Threonine Kinases - genetics
/ Protein-Serine-Threonine Kinases - metabolism
