Mitochondrial dysfunction and Huntington disease

Neuroscience Bulletin

Volumn 22, Issue 2, 2006, Pages 129-136

  Zhang, Wei Yan ^a   Gu, Zhen Lun ^b   Liang, Zhong Qin ^b   Qin, Zheng Hong ^b  

^a SOOCHOW UNIVERSITY   (China)

^b MEDICAL COLLEGE OF SOOCHOW UNIVERSITY   (China)

Author keywords

Huntingtin; Huntington disease; Mitochondrial dysfunction

Indexed keywords

CREATINE;

BRAIN REGION; CLINICAL TRIAL; DISORDERS OF MITOCHONDRIAL FUNCTIONS; GENETIC CODE; GENETIC IDENTIFICATION; HISTOPATHOLOGY; HUMAN; HUNTINGTON CHOREA; NONHUMAN; PATHOGENESIS; REVIEW;

EID: 33947133201     PISSN: 16737067     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Review

References (66)

1. Fridovich I. The biology of oxygen radicals. Science, 1978, 201(4359):875-880.
2. Papa S, Skulachev VP. Reactive oxygen species, mitochondria, apoptosis and aging. Mol Cell Biochem, 1997, 174(1-2):305-319.
3. Chance B, Sies H, Boveris A. Hydroperoxide metabolism in mammalian organs. Physiol Rev, 1979, 59(3):527-605.
4. Halliwell B, Gutteridge JM. Role of free radicals and catalytic metal ions in human disease: an overview. Methods Enzymol, 1990, 186:1-85.
5. Groves JT. Peroxynitrite: reactive, invasive and enigmatic. Curr Opin Chem Biol, 1999, 3(2):226-235.
6. Szabo C, Ohshima H. DNA damage induced by peroxynitrite: subsequent biological effects. Nitric Oxide, 1997, 1(5):373-385.
7. O'Connor M, Salzman AL, Szabo C. Role of peroxynitrite in the protein oxidation and apoptotic DNA fragmentation in vascular smooth muscle cells stimulated with bacterial lipopolysaccharide and interferon-gamma. Shock, 1997, 8(6):439-443.
8. Lin KT, Xue JY, Lin MC, et al. Peroxynitrite induces apoptosis of HL-60 cells by activation of a caspase-3 family protease. Am J Physiol, 1998, 274(4): C855-860.
9. Liu GY, Chen KJ, Lin-Shiau SY, et al. Peroxyacetyl nitrateinduced apoptosis through generation of reactive oxygen species in HL-60 cells. Mol Carcinog, 1999, 25(3):196-206.
10. Chung HT, Pae HO, Choi BM, et al. Nitric oxide as a bioregulator of apoptosis. Biochem Biophys Res Co, 2001, 282 (5):1075 1079.
11. Anderson S, Bankier AT, Barrell BG, et al. Sequence and organization of the human mitochondrial genome. Nature, 1981, 290(5806):457-465.
12. Alston TA, Mela L, Bright HJ. 3-Nitropropionate, the toxic substance of Indigofera, is a suicide inactivator of succinate dehydrogenase. Proc Natl Acad Sci USA, 1977, 74(9):3767-3771.
13. He F, Zhang S, Qian F, et al. Delayed dystonia with striatal CT lucencies induced by a mycotoxin (3-nitropropionic acid). Neurology, 1995,45(12):2178-2183.
14. Beal MF, Brouillet E, Jenkins BG, et al. Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid. J Neurosci, 1993, 13(10):4181-4192.
15. Bizat N, Hermel JM, Boyer F, et al. Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease. J Neurosci, 2003,23(12):5020-5030.
16. Leventhal L, Sortwell CE, Hanbury R, et al. Cyclosporin A protects striatal neurons in vitro and in vivo from 3-nitropropionic acid toxicity. J Comp Neurol,2000, 425(4):471-478.
17. 2+ and respiratory chain complex II inhibition. J Neurochem, 2004, 90(5):1025-1035.
18. Galas MC, Bizat N, Cuvelier L, et al. Death of cortical and striatal neurons induced by mitochondrial defect involves differential molecular mechanisms. Neurobiol Dis,2004,15(1):152-159.
19. Almeida S, Domingues A, Rodrigues L, et al. FK506 prevents mitochondrial-dependent apoptotic cell death induced by 3-nitropropionic acid in rat primary cortical cultures. Neurobiol Dis,2004,17(3):435-444.
20. Brouillet E, Hantraye P, Ferrante RJ, et al. Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates. Proc Natl Acad Sci USA,1995,92(15):7105-7109.
21. Palfi S, Ferrante RJ, Brouillet E, et al. Chronic 3-nitropropionic acid treatment in baboons replicates the cognitive and motor deficits of Huntington's disease. J Neurosci,1996,16(9):3019-3025.
22. Beal MF, Brouillet E, Jenkins B, et al. Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate. J Neurochem,1993,61(3):1147-1150.
23. Gusella JF, Wexler NS, Conneally PM, et al. A polymorphic DNA marker genetically linked to Huntington's disease. Nature,1983,306(5940):234- 238.
24. The Huntington's Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is unstable in Huntington's disease chromosomes. Cell,1993,72(6):971 983.
25. Duyao M, Ambrose C, Myers R, et al. Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet, 1993,4(4):387-392.
26. Goldberg YP, Kremer B, Andrew SE, et al. Molecular analysis of new mutations for Huntington's disease: Intermediate alleles and sex of origin effects. Nat Genet,1993,5(2):174-179.
27. Arnould T, Vankoningsloo S, Renard P, et al. CREB activation induced by mitochondrial dysfunction is a new signaling pathway that impairs cell proliferation. EMBO J, 2002,21(1-2):53-63.
28. Gauthier LR, Charrin BC, Borrell-Pages M. et al. Huntingtin controls neurotrophic support and survival of neurons by enhancing BD-NF vesicular transport along microtubules. Cell,2004,118(1):127 138.
29. Qin ZH, Gu ZL. Huntingtin processing in pathogenesis of Huntington disease. Acta Pharmacol Sin, 2004, 25(10): 1243-1249.
30. Ruan Q, Lesort M, MacDonald ME, et al. Striatal cells from mutant huntingtin knock-in mice are selectively vulnerable to mitochondrial complex II inhibitor-induced cell death through a non-apoptotic pathway. Hum Mol Genet,2004, 13(7):669 681.
31. Bae BI, Xu H, Igarashi S, et al. p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease. Neuron, 2005, 47(1): 29-41.
32. Yu ZX, Li SH, Evans J, et al. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci, 2003,23(6):2193-2202.
33. Chang DT, Rintoul GL, Pandipati S, et al. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol Dis,2006 [Epub ahead of print].
34. 2+-dependent depolarization of rat and human mitochondria: Relevance to Huntington's disease. Arch Biochem Biophys, 2003,410(1):1-6.
35. Brustovetsky N, LaFrance R, Purl KJ, et al. Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's disease. J Neurochem,2005,93 (6):1361-1370.
36. Panov AV, Gutekunst CA, Leavitt BR, et al. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci,2002,5(8):731-736.
37. Choo YS, Johnson GV, MacDonald M, et al. Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet,2004,13(14): 1407-1420.
38. 2+-induced permeability transition in human lymphoblastoid cell mitochondria from normal and Huntington's disease individuals. Mol Cell Biochem,2005,269(1-2):143-152.
39. Puranam KL, Wu GH, Strittmatter WJ, et al. Polyglutamine expansion inhibits respiration by increasing reactive oxygen species in isolated mitochondria. Biochem Biophys Res Co,2006,341(2):607-613.
40. Choo YS, Mao Z, Gohnson GVm, et al. Increased glutathione levels in cortical and striatal mitochondria of the R6/2 Huntington's disease mouse model. Neurosci Lett,2005,386(1):63-68.
41. Vonsattel JP, Myers RH, Stevens TJ, et al. Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurol, 1985,44(6):559-577.
42. Gu M, Gash MT, Mann VM, et al. Mitochondrial defect in Huntington's disease caudate nucleus. Ann Neurol,1996,39(3):385-389.
43. 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(5):646-653.
44. Dawbarn D, De Quidt ME, Emson PC. Survival of basal ganglia neuropeptide Y-somatostatin neurones in Huntington's disease. Brain Res, 1985,340(2):251-260.
45. Ferrante RJ, Kowall NW, Beal MF, et al. Selective sparing of a class of striatal neurons in Huntington's disease. Science, 1985,230(4725):561- 563.
46. Ferrante RJ, Kowall NW, Beal MF, et al. Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease. J Neuropathol Exp Neurol, 1987,46(1):12-27.
47. Kowall NW, Ferrante RJ, Martin JB. Patterns of cell loss in Huntington's disease. Trends Neurosci,1987,10:24-29.
48. Cicchetti F, Parent A. Striatal interneurons in Huntington's disease: selective increase in the density of calretinin-immunoreactive medium-sized neurons. Mov Disord ,1996,11(6):619-626.
49. Schapira AH. Mitochondrial function in Huntington's disease: clues for pathogenesis and prospects for treatment. Ann Neurol, 1997,41(2):141-142.
50. Cooper JM, Schapira AH. Mitochondrial dysfunction in neurodegeneration. J Bioenerg Biomembr,1997,29(2):175-183.
51. Beal MF. Mitochondrial dysfunction in neurodegenerative diseases. Biochim Biophys Acta,1998,1366(1-2):211-223.
52. Fiskum G, Murphy AN, Beal MF. Mitochondria in neurodegeneration: acute ischemia and chronic neurodegenerative diseases. J Cereb Blood Flow Metab,1999,19(4):351-369.
53. 1H NMR spectroscopy. Neurology,1993, 43(12):2689-2695.
54. 10. Ann Neurol,1997,41(2):160-165.
55. Lodi R, Schapira AH, Manners D, et al. Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy. Ann Neurol,2000,48(1):72-76.
56. Gines S, Seong IS, Fossale E, et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet,2003,12(5):497-508.
57. Gaba AM, Zhang K, Marder K, et al. Enery balance in early-stage Huntington disease. Am J Clin Nutr, 2005, 81(6): 1335-41.
58. Saft C, zange J, Andrich J, et al. Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease. Mov Disord, 2005, 20(6):674-679.
59. Qin ZH, Wang J, Gu ZL. Development of novel therapies for huntington's disease: hope and challenge. Acta Pharmacol Sin,2005 26(2):129-142.
60. Ferrante RJ, Andreassen OA, Jenkins BG, et al. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci,2000,20(12):4389 4397.
61. Tabrizi SJ, Blamire AM, Manners DN, et al. High-dose creatine therapy for Huntington disease: A 2-year clinical and MRS study. Neurology,2005,64(9):1655-61.
62. Peyser CE, Folstein M, Chase GA, et al. Trial of d-alpha-tocopherol in Huntington's disease. Am J Psychiatry, 1995, 152(12):1771-1775.
63. Ranen NG, Peyser CE, Coyle JT, et al. A controlled trial of idebenone in Huntington's disease. Mov Disord, 1996, 11(5):549-554.
64. Huntington Study Group. A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology, 2001, 57(3):397-404.
65. Ferrante RJ, Andreassen OA, Dedeoglu A, et al. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci, 2002, 22(5):1592-1599.
66. Schilling G, Coonfield ML, Ross CA, et al. Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model. Neurosci Lett, 2001, 315(3):149-153.