D-β-hydroxybutyrate is protective in mouse models of Huntington's disease

• Published in: PloS one Vol. 6; no. 9; p. e24620
• Main Authors: Lim, Soyeon, Chesser, Adrianne S, Grima, Jonathan C, Rappold, Phillip M, Blum, David, Przedborski, Serge, Tieu, Kim
• Format: Journal Article
• Language: English
• Published: United States Public Library of Science 12.09.2011; Public Library of Science (PLoS)
• Subjects: 3-Hydroxybutyric Acid - therapeutic use; 3-Nitropropionic acid; Abnormalities; Acetylation - drug effects; Activation; Animal cognition; Animal models; Animals; Bioenergetics; Biology; Chemical compounds; Deacetylation; Dehydrogenases; Diet; Epigenetics; Feasibility studies; Genetic disorders; Histone deacetylase; Histones - metabolism; Huntingtin; Huntington Disease - drug therapy; Huntington's disease; Huntingtons disease; Immunoblotting; Lesions; Male; Medicine; Metabolism; Mice; Mice, Inbred C57BL; Mitochondria; Neostriatum; Neurodegeneration; Neurology; Neurons; Neuroprotection; Neurotoxicity; Nitro Compounds - therapeutic use; Parkinson's disease; PC12 Cells; Pharmacology; Pheochromocytoma cells; Polyglutamine; Propionates - therapeutic use; Proteins; Rats; Rodents; Transgenic mice; Trinucleotide repeat diseases; France; New York; United States > US

Abnormalities in mitochondrial function and epigenetic regulation are thought to be instrumental in Huntington's disease (HD), a fatal genetic disorder caused by an expanded polyglutamine track in the protein huntingtin. Given the lack of effective therapies for HD, we sought to assess the neur...