Given that C. elegans is a robust translational model in which to not only dissect disease mechanisms, but also to evaluate candidate therapies for mitochondrial disease (24, 36), we sought to evaluate whether the significantly impaired fecundity, development, growth, and neurologic and/or muscular activity of fbxl-1(ok3741) worms could be rescued with empiric mitochondrial disease therapies (37). Here, SKP2 is linked to inborn mitochondrial metabolism disorder.