Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

Annals of Neurology

Volumn 50, Issue 1, 2001, Pages 112-116

  Andreassen, Ole A ^a   Ferrante, Robert J ^b,c   Huang, Hsueh Meei ^d   Dedeoglu, Alpaslan ^a   Park, Larry ^d   Ferrante, Kimberly L ^d   Kwon, Jennifer ^d   Borchelt, David R ^e   Ross, Christopher A ^e   Gibson, Gary E ^f   Beal, M Flint ^d  

^a HARVARD MEDICAL SCHOOL   (United States)

^b VETERANS AFFAIRS MEDICAL CENTER   (United States)

^c BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d New York Presbyterian Hospital ^*  (United States)

^e JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^f BURKE MEDICAL RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DICHLOROACETIC ACID; LACTIC ACID; PYRUVATE DEHYDROGENASE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BODY WEIGHT; CONTROLLED STUDY; DIABETES MELLITUS; DRUG EFFECT; ENZYME ACTIVITY; HUNTINGTON CHOREA; MALE; MOTOR PERFORMANCE; MOUSE; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; STRIATE CORTEX; TRANSGENIC MOUSE;

EID: 0034955984     PISSN: 03645134     EISSN: None     Source Type: Journal    
DOI: 10.1002/ana.1085     Document Type: Article

References (21)

1. Energetics in the pathogenesis of neurodegenerative diseases
2. 1H NMR spectroscopy
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4. Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy
5. Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization
6. Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates
7. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease
8. Treatment of congenital lactic acidosis with dichloroacetate
9. Stabilization of the pyruvate dehydrogenase E1a subunit by dichloroacetate
10. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
11. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
12. The role of cytosolic free calcium in the regulation of pyruvate dehydrogenase in synaptosomes
13. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
14. Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene
15. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
16. Exponential decrease over time in N-acetylaspartate levels in the absence of neuronal loss and increases in glutamine and glucose in transgenic Huntington's disease mice
17. Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
18. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
19. Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain
20. Improvement of lesions shown on MRI and CT scan by administration of dichloroacetate in patients with Leigh syndrome
21. Effects of dichloroacetate in three patients with MELAS