Parkinson disease is the second most common neurodegenerative disorder after Alzheimer disease. Sufferers experience motor skill abnormalities including tremor, muscle stiffness, and unstable voluntary movements and posture. The main pathological feature of the Parkinson brain is the loss of dopaminergic neurons.
Reported in an article in the September 15 issue of the Journal of Clinical Investigation, Przedborski and colleagues administered the neurotoxin MPTP to mice, which caused dopaminergic neurodegeneration and deficits in the mitochondrial electron transport chain reminiscent of Parkinson disease. Using this model of disease, the authors showed that the infusion of the ketone body D-beta-HB restored mitochondrial respiration and protected against MPTP-induced neurodegeneration and motor deficits. The study supports a critical role for mitochondrial defect in Parkinson disease.
Ketone bodies are already successfully used in the treatment of epilepsy. They are also able to penetrate the blood-brain barrier that often prevents potentially beneficial drugs from entering the brain.
D-beta-HB may therefore be considered as a novel form of neuroprotective therapy in the treatment of Parksinson disease.
TITLE: D-beta-Hydroxybutyrate rescues mitochondrial respiration and mitigates features of Parkinson disease
AUTHOR CONTACT:
Serge Przedborski
Columbia University, New York, New York, USA.
Phone: (212) 305-1540
Fax: (212) 305-5450
E-mail: SP30@columbia.edu
View the PDF of this article at: http://www.jci.org/cgi/content/full/112/6/892
Journal of Clinical Investigation