TY - JOUR AU - Tieu, Kim AU - Perier, Celine AU - Caspersen, Casper AU - Teismann, Peter AU - Wu, Du-Chu AU - Yan, Shi-Du AU - Naini, Ali AU - Vila, Miquel AU - Jackson-Lewis, Vernice AU - Ramasamy, Ravichandran AU - Przedborski, Serge T1 - D-β-Hydroxybutyrate rescues mitochondrial respiration and mitigates features of Parkinson disease PY - 2003/09/15/ AB - Parkinson disease (PD) is a neurodegenerative disorder characterized by a loss of the nigrostriatal dopaminergic neurons accompanied by a deficit in mitochondrial respiration. 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is a neurotoxin that causes dopaminergic neurodegeneration and a mitochondrial deficit reminiscent of PD. Here we show that the infusion of the ketone body D-β-hydroxybutyrate (DβHB) in mice confers partial protection against dopaminergic neurodegeneration and motor deficits induced by MPTP. These effects appear to be mediated by a complex II–dependent mechanism that leads to improved mitochondrial respiration and ATP production. Because of the safety record of ketone bodies in the treatment of epilepsy and their ability to penetrate the blood-brain barrier, DβHB may be a novel neuroprotective therapy for PD. JF - The Journal of Clinical Investigation JA - J Clin Invest SN - 0021-9738 DO - 10.1172/JCI18797 VL - 112 IS - 6 UR - https://doi.org/10.1172/JCI18797 SP - 892 EP - 901 PB - The American Society for Clinical Investigation ER -