Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse

Annals of Neurology

Volumn 47, Issue 1, 2000, Pages 80-86

  Tabrizi, S J ^a   Workman, J ^a   Hart, P E ^a   Mangiarini, L ^b   Mahal, A ^b   Bates, G ^b   Cooper, J M ^a   Schapira, A H V ^a,c  

^a ROYAL FREE AND UNIVERSITY COLLEGE MEDICAL SCHOOL   (United Kingdom)

^b KING'S COLLEGE LONDON   (United Kingdom)

^c UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

3 NITROTYROSINE; FREE RADICAL; NITRIC OXIDE SYNTHASE;

ANIMAL CELL; ANIMAL EXPERIMENT; ARTICLE; DEGENERATIVE DISEASE; ENERGY METABOLISM; ENZYME ACTIVITY; FROZEN SECTION; GENETIC DAMAGE; GENETIC DISORDER; HUNTINGTON CHOREA; IMMUNOBLOTTING; MALE; MITOCHONDRIAL RESPIRATION; MOUSE; MUSCLE ATROPHY; NERVE CELL NECROSIS; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; RECEPTOR BINDING; RESPIRATORY CHAIN; TRANSGENE;

ANIMALS; BRAIN; DISEASE MODELS, ANIMAL; HUNTINGTON DISEASE; IMMUNOHISTOCHEMISTRY; MICE; MICE, TRANSGENIC; MITOCHONDRIAL MYOPATHIES;

EID: 0033982887     PISSN: 03645134     EISSN: None     Source Type: Journal    
DOI: 10.1002/1531-8249(200001)47:1<80::AID-ANA13>3.0.CO;2-K     Document Type: Article

References (42)

1. Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 1993;72: 971-983
2. Trottier Y, Devys D, Imbert G, et al. Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form. Nat Genet 1995;10:104-110
3. Trottier Y, Lutz Y, Stevanin G, et al. Polyglutamine expansion as a pathological epitope in Huntington's disease and 4 dominant cerebellar ataxias. Nature 1995;378:402-406
4. Von Sattel JP, Myers RH, Stevens TJ, et al. Neuropathological classification of Huntington's disease. Neuropathol Exp Neurol 1985;44:559-577
5. Becher MW, Kotzuk JA, Sharp AH, et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian arrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol Dis 1998; 4:387-397
6. DiFiglia M, Sapp E, Chase KO, et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1998;277:1990-1993
7. Gu M, Cooper JM, Gash M, et al. Mitochondrial defect in Huntington's disease caudate nucleus. Ann Neurol 1996;39: 385-389
8. Browne SE, Bowling AC, MacGarvey U, et al. Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. Ann Neurol 1997;41: 646-653
9. Tabrizi SJ, Cleeter M, Xuereb J, et al. Biochemical abnormalities and excitotoxicity in Huntington's disease brain. Ann Neurol 1999;45:25-32
10. Brouillet E, Jenkins BG, Hyman BT. Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid. J Neurochem 1993;60:356-359
11. Kuwert T, Lange HW, Langer K-J, et al. Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease. Brain 1990;113:1405-1423
12. 1H NMR spectroscopy. Neurology 1993;43: 2689-2695
13. 1H NMR spectroscopy studies in Huntington's disease: correlations with CAG repeat numbers. Neurology 1998;5:1357-1363
14. Hoang TQ, Dubowitz J, Moats R, et al. Quantitative proton-decoupled P-31 MRS and H-1 MRS in the evaluation of Huntington's and Parkinson's disease. Neurology 1998;50:1033-1040
15. Parker WD, Boyson SJ, Luder AS, Parks JK. Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's disease. Neurology 1990;40:1231-1234
16. 10. Ann Neurol 1997;41:160-165
17. Arenas J, Campos Y, Ribacoba R. Complex I defect in muscle from patients with Huntington's disease. Ann Neurol 1998;43: 397-400
18. Hausladen A, Fridovich I. Superoxide and peroxynitrite inactivates aconitases, but nitric oxide does not. J Biol Chem 1994; 269:29405-29408
19. Patel M, Day BJ, Crapo JD, et al. Requirement for superoxide in excitotoxic cell death. Neuron 1996;16:345-355
20. Dawson VL, Dawson TM, London ED, et al. Nitric oxide mediates glutamate neurotoxicity in primary cortical cultures. Proc Natl Acad Sci USA 1991;88:6368-6371
21. Dawson VL, Dawson TM, Bartley DA, et al. Mechanisms of nitric oxide-mediated neurotoxicity in primary brain cultures. J Neurosci 1993;13:2651-2661
22. Beal MF, Ferrante RJ, Swartz KJ, Kowall NW. Chronic quinolinic acid lesions in rats closely mimic Huntington's disease. J Neurosci 1991;11:1649-1659
23. Novelli A, Reilly JA, Lysko PG, Henneberry RC. Glutamate becomes neurotoxic via the N-methyl-D-aspartate receptor when intracellular energy levels are reduced. Brain Res 1998; 451:205-212
24. Beal MF. Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses? Ann Neurol 1992;31:119-130
25. Bolaños JP, Almeida A, Stewart V, et al. Nitric oxide-mediated mitochondrial damage in the brain: mechanisms and implications for neurodegenerative diseases. J Neurochem 1997;68: 2227-2240
26. Mangiarini L, Sathasivam K, Seller M, et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996; 87:493-506
27. Davies SW, Turmaine M, Cozens BA, et al. From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease. Philos Trans Roy Soc Lond B Biol Sci 1999;354:971-979
28. Cha JHJ, Kosinski CM, Kerner JA, et al. Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene. Proc Natl Acad Sci USA 1998;95:6480-6485
29. Calabresi P, Pisani A, Mercuri NB, Bernardi G. The cortico-spinal projection: from synaptic plasticity to dysfunctions of the basal ganglia. Trends Neurosci 1996;19:19-24
30. Petralia RS, Wang YX, Niedzielski AS, Wenthold RJ. The metabotropic glutamate receptors, mGluR2 and mGluR3, show unique postsynaptic, presynaptic and glial localizations. Neuroscience 1996;71:949-976
31. Levine MS, Chesselet MF, Koppel A, et al. Enhanced sensitivity to glutamate receptor activation in mouse models of Huntington's disease. Soc Neurosci Abstr 1998;24:972 (Abstract)
32. Bogdanov M, Ferrante RJ, Kuemmerle S, et al. Increased vulnerability to 3-nitropropionic acid in an animal model of Huntington's disease. J Neurochem 1998;71:2642-2644
33. Schapira AHV, Cooper JM, Dexter D, et al. Mitochondrial complex I deficiency in Parkinson's disease. J Neurochem 1990; 54:823-827
34. Taanman J-W, Bodnar AG, Cooper JM, et al. Molecular mechanisms in mitochondrial DNA depletion syndrome. Hum Mol Genet 1997;6:935-942
35. Dubowitz V. Muscle biopsy. London: Balliere-Tindall, 1985: 35-36
36. Wong-Riley MTT. Cytochrome oxidase: an endogenous metabolic marker for neuronal activity. Trends Neurosci 1989;12: 94-101
37. Schapira AHV. Mitochondrial dysfunction in neurodegenerative disorders. Biochim Biophys Acta 1998;1366:225-233
38. Murphy S, Grzybicki D. Glial NO: normal and pathological roles. Neuroscientist 1996;2:90-99
39. Beckman JS. Oxidative damage and tyrosine nitration from peroxynitrite. Chem Res Toxicol 1996;9:836-844
40. Coyle JT, Puttfarcken P. Oxidative stress, glutamate, and neurodegenerative disorders. Science 1993;262:689-695
41. Schilling G, Becher MW, Sharp AH, et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 1999;8:397-407
42. Sathasivam K, Hobbs C, Turmaine M, et al. Formation of polyglutamine inclusions in non-CNS tissue. Hum Mol Genet 1999;8:813-822