메뉴 건너뛰기




Volumn 157, Issue 1, 1999, Pages 1-18

Recent advances on the pathogenesis of Huntington's disease

Author keywords

Excitotoxicity; Huntingtin; Huntington's disease; Neuropathology; Pathogenesis; Striatum; Transg enic disease model

Indexed keywords

CALMODULIN; CASPASE; HUNTINGTIN; UBIQUITIN;

EID: 0032912386     PISSN: 00144886     EISSN: None     Source Type: Journal    
DOI: 10.1006/exnr.1998.7006     Document Type: Review
Times cited : (132)

References (168)
  • 1
    • 0028836862 scopus 로고
    • Genetics and molecular biology of Huntington's disease
    • Albin R. L., Tagle D. A. Genetics and molecular biology of Huntington's disease. Trends Neurosci. 18:1995;11-14.
    • (1995) Trends Neurosci. , vol.18 , pp. 11-14
    • Albin, R.L.1    Tagle, D.A.2
  • 2
    • 0032479940 scopus 로고    scopus 로고
    • 3-Nitroproprionic acid's lethal triplet: Cooperative pathways of neurodegeneration
    • Alexi T., Hughes P. E., Faull R. L., Williams C. E. 3-Nitroproprionic acid's lethal triplet: Cooperative pathways of neurodegeneration. Neuroreport. 9:1998;R57-R64.
    • (1998) Neuroreport , vol.9
    • Alexi, T.1    Hughes, P.E.2    Faull, R.L.3    Williams, C.E.4
  • 4
    • 0029392854 scopus 로고
    • HEAT repeats in the Huntington's disease protein
    • Andrade M. A., Bork P. HEAT repeats in the Huntington's disease protein. Nature Genet. 11:1995;115-116.
    • (1995) Nature Genet. , vol.11 , pp. 115-116
    • Andrade, M.A.1    Bork, P.2
  • 6
    • 0028793257 scopus 로고
    • Glutamate-induced neuronal death: A succession of necrosis or apoptosis depending on mitochondrial function
    • Ankarcrona M., Dypbukt J. M., Bonfoco E., Zhivotovsky B., Orrenius S., Lipton S. A., Nicotera P. Glutamate-induced neuronal death: A succession of necrosis or apoptosis depending on mitochondrial function. Neuron. 15:1995;961-973.
    • (1995) Neuron , vol.15 , pp. 961-973
    • Ankarcrona, M.1    Dypbukt, J.M.2    Bonfoco, E.3    Zhivotovsky, B.4    Orrenius, S.5    Lipton, S.A.6    Nicotera, P.7
  • 7
    • 0030574021 scopus 로고    scopus 로고
    • Calcineurin and mitochondrial function in glutamate induced neuronal apoptosis
    • Ankarcrona M., Dypbukt J. M., Orrenius S., Nicotera P. Calcineurin and mitochondrial function in glutamate induced neuronal apoptosis. FEBS Lett. 394:1996;321-324.
    • (1996) FEBS Lett. , vol.394 , pp. 321-324
    • Ankarcrona, M.1    Dypbukt, J.M.2    Orrenius, S.3    Nicotera, P.4
  • 9
    • 0030708576 scopus 로고    scopus 로고
    • Changes of NMDA receptor subunit (NR1, NR2B) and glutamate transporter (GLT1) mRNA expression in Huntington's disease-An in situ hybridization study
    • Arzberger T., Krampel K., Leimgruber S., Weindl A. Changes of NMDA receptor subunit (NR1, NR2B) and glutamate transporter (GLT1) mRNA expression in Huntington's disease-An in situ hybridization study. J. Neuropathol. Exp. Neurol. 56:1997;440-454.
    • (1997) J. Neuropathol. Exp. Neurol. , vol.56 , pp. 440-454
    • Arzberger, T.1    Krampel, K.2    Leimgruber, S.3    Weindl, A.4
  • 11
    • 0030612119 scopus 로고    scopus 로고
    • Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease
    • Augood S. J., Faull R. L., Emson P. C. Dopamine D1 and D2 receptor gene expression in the striatum in Huntington's disease. Ann. Neurol. 42:1997;215-221.
    • (1997) Ann. Neurol. , vol.42 , pp. 215-221
    • Augood, S.J.1    Faull, R.L.2    Emson, P.C.3
  • 13
    • 0022446150 scopus 로고
    • Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid
    • Beal M. F., Kowall N. W., Ellison D. W., Mazurek M. F., Swartz K. J., Martin J. B. Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature. 321:1986;168-171.
    • (1986) Nature , vol.321 , pp. 168-171
    • Beal, M.F.1    Kowall, N.W.2    Ellison, D.W.3    Mazurek, M.F.4    Swartz, K.J.5    Martin, J.B.6
  • 14
    • 0026043565 scopus 로고
    • Chronic quinolinic acid lesions in rats closely resemble Huntington's disease
    • Beal M. F., Ferrante R. J., Swartz K. J., Kowall N. W. Chronic quinolinic acid lesions in rats closely resemble Huntington's disease. J. Neurosci. 11:1989;1649-1959.
    • (1989) J. Neurosci. , vol.11 , pp. 1649-1959
    • Beal, M.F.1    Ferrante, R.J.2    Swartz, K.J.3    Kowall, N.W.4
  • 15
    • 0026584524 scopus 로고
    • Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses
    • Beal M. F. Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses. Ann. Neurol. 31:1992;119-130.
    • (1992) Ann. Neurol. , vol.31 , pp. 119-130
    • Beal, M.F.1
  • 16
    • 0027204154 scopus 로고
    • Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate
    • Beal M. F., Brouillet E., Jenkins B., Henshaw R., Rosen B., Hyman B. T. Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate. J. Neurochem. 61:1993;1147-1150.
    • (1993) J. Neurochem. , vol.61 , pp. 1147-1150
    • Beal, M.F.1    Brouillet, E.2    Jenkins, B.3    Henshaw, R.4    Rosen, B.5    Hyman, B.T.6
  • 17
    • 0027477946 scopus 로고
    • Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases
    • Beal M. F., Hyman B. T., Koroshetz W. Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases. Trends Neurosci. 16:1993;125-131.
    • (1993) Trends Neurosci. , vol.16 , pp. 125-131
    • Beal, M.F.1    Hyman, B.T.2    Koroshetz, W.3
  • 18
    • 0029125857 scopus 로고
    • Aging, energy and oxidative stress in neurodegenerative diseases
    • Beal M. F. Aging, energy and oxidative stress in neurodegenerative diseases. Ann. Neurol. 38:1995;357-366.
    • (1995) Ann. Neurol. , vol.38 , pp. 357-366
    • Beal, M.F.1
  • 19
    • 0031918640 scopus 로고    scopus 로고
    • Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions andIT15
    • Becher M. W., Kotzuk J. A., Sharp A. H., Davies S. W., Bates G. P., Price D. L., Ross C. A. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions andIT15. Neurobiol. Dis. 4:1998;387-397.
    • (1998) Neurobiol. Dis. , vol.4 , pp. 387-397
    • Becher, M.W.1    Kotzuk, J.A.2    Sharp, A.H.3    Davies, S.W.4    Bates, G.P.5    Price, D.L.6    Ross, C.A.7
  • 22
    • 0019425263 scopus 로고
    • Neuropathological changes of the nucleus accumbens in Huntington's chorea
    • Bots G. Th. A. M., Bruyn G. W. Neuropathological changes of the nucleus accumbens in Huntington's chorea. Acta Neuropathol. 55:1987;21-22.
    • (1987) Acta Neuropathol. , vol.55 , pp. 21-22
    • Bots, G.th.a.m.1    Bruyn, G.W.2
  • 23
    • 0029925184 scopus 로고    scopus 로고
    • Trinucleotide repeat length and clinical progression in Huntington's disease
    • Brandt J., Bylsma F. W., Gross R., Stine O. C., Ranen N., Ross C. A. Trinucleotide repeat length and clinical progression in Huntington's disease. Neurology. 46:1996;527-531.
    • (1996) Neurology , vol.46 , pp. 527-531
    • Brandt, J.1    Bylsma, F.W.2    Gross, R.3    Stine, O.C.4    Ranen, N.5    Ross, C.A.6
  • 24
    • 0029614765 scopus 로고
    • Neural apoptosis
    • Bredesen D. E. Neural apoptosis. Ann. Neurol. 38:1995;839-851.
    • (1995) Ann. Neurol. , vol.38 , pp. 839-851
    • Bredesen, D.E.1
  • 25
    • 0025941639 scopus 로고
    • Nitric oxide synthase protein and mRNA are discretely localized in neuronal populations of the mammalian CNS together with NADPH diaphorase
    • Bredt D. S., Glatt C. E., Hwang P. M., Fotuhi M., Dawson T. M., Snyder S. H. Nitric oxide synthase protein and mRNA are discretely localized in neuronal populations of the mammalian CNS together with NADPH diaphorase. Neuron. 7:1991;615-624.
    • (1991) Neuron , vol.7 , pp. 615-624
    • Bredt, D.S.1    Glatt, C.E.2    Hwang, P.M.3    Fotuhi, M.4    Dawson, T.M.5    Snyder, S.H.6
  • 27
    • 0032581167 scopus 로고    scopus 로고
    • Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum
    • Butterworth N. J., Williams L., Bullock J. Y., Love D. R., Faull R. L., Dragunow M. Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum. Neuroscience. 87:1998;49-53.
    • (1998) Neuroscience , vol.87 , pp. 49-53
    • Butterworth, N.J.1    Williams, L.2    Bullock, J.Y.3    Love, D.R.4    Faull, R.L.5    Dragunow, M.6
  • 28
    • 0027301377 scopus 로고
    • Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: A pathological correlate of dementia
    • Cammarata S., Caponnetto C., Tabaton M. Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: A pathological correlate of dementia. Neurosci. Lett. 156:1993;96-98.
    • (1993) Neurosci. Lett. , vol.156 , pp. 96-98
    • Cammarata, S.1    Caponnetto, C.2    Tabaton, M.3
  • 33
  • 34
    • 0017167057 scopus 로고
    • Lesion of striatal neurones with kainic acid provides a model for Huntington's chorea
    • Coyle J. T., Schwarcz R. Lesion of striatal neurones with kainic acid provides a model for Huntington's chorea. Nature. 263:1976;244-246.
    • (1976) Nature , vol.263 , pp. 244-246
    • Coyle, J.T.1    Schwarcz, R.2
  • 35
    • 0027686249 scopus 로고
    • Oxidative stress, glutamate, and neurodegenerative disorders
    • Coyle J. T., Puttfarcken P. Oxidative stress, glutamate, and neurodegenerative disorders. Science. 262:1993;689-695.
    • (1993) Science , vol.262 , pp. 689-695
    • Coyle, J.T.1    Puttfarcken, P.2
  • 37
    • 0032501740 scopus 로고    scopus 로고
    • Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions
    • Davies S. W., Beardsall K., Turmaine M., DiFiglia M., Aronin N., Bates G. P. Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions. Lancet. 351:1998;131-133.
    • (1998) Lancet , vol.351 , pp. 131-133
    • Davies, S.W.1    Beardsall, K.2    Turmaine, M.3    Difiglia, M.4    Aronin, N.5    Bates, G.P.6
  • 38
    • 0025823405 scopus 로고
    • Nitric oxide synthase and neuronal NADPH diaphorase are identical in brain and peripheral tissue
    • Dawson T. M., Bredt D. S., Fotuhi M., Hwang P. M., Snyder S. H. Nitric oxide synthase and neuronal NADPH diaphorase are identical in brain and peripheral tissue. Proc. Natl. Acad. Sci. USA. 88:1991;7797-7801.
    • (1991) Proc. Natl. Acad. Sci. USA , vol.88 , pp. 7797-7801
    • Dawson, T.M.1    Bredt, D.S.2    Fotuhi, M.3    Hwang, P.M.4    Snyder, S.H.5
  • 39
    • 0027178135 scopus 로고
    • Mechanisms of nitric oxide-mediated neurotoxicity in primary brain cultures
    • Dawson V. L., Dawson T. M., Bartley D. A., Uhl G. R., Snyder S. H. Mechanisms of nitric oxide-mediated neurotoxicity in primary brain cultures. J. Neurosci. 13:1993;2651-2661.
    • (1993) J. Neurosci. , vol.13 , pp. 2651-2661
    • Dawson, V.L.1    Dawson, T.M.2    Bartley, D.A.3    Uhl, G.R.4    Snyder, S.H.5
  • 40
    • 0029927787 scopus 로고    scopus 로고
    • Resistance to neurotoxicity in cortical cultures from neuronal nitric oxide synthase-deficient mice
    • Dawson V. L., Kizushi V. M., Huang P. L., Snyder S. H., Dawson T. M. Resistance to neurotoxicity in cortical cultures from neuronal nitric oxide synthase-deficient mice. J. Neurosci. 16:1996;2479-2487.
    • (1996) J. Neurosci. , vol.16 , pp. 2479-2487
    • Dawson, V.L.1    Kizushi, V.M.2    Huang, P.L.3    Snyder, S.H.4    Dawson, T.M.5
  • 41
    • 0029816724 scopus 로고    scopus 로고
    • Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation
    • De Rooij K. E., Dorsman J. C., Smoor M. A., Den Dunnen J. T., Van Ommen G.-J. B. Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation. Hum. Mol. Genet. 5:1996;1093-1099.
    • (1996) Hum. Mol. Genet. , vol.5 , pp. 1093-1099
    • De Rooij, K.E.1    Dorsman, J.C.2    Smoor, M.A.3    Den Dunnen, J.T.4    Van Ommen, G.-J.B.5
  • 42
    • 0029302244 scopus 로고
    • Apoptosis in the brain
    • Dickson D. W. Apoptosis in the brain. Am. J. Pathol. 146:1995;1040-1044.
    • (1995) Am. J. Pathol. , vol.146 , pp. 1040-1044
    • Dickson, D.W.1
  • 44
    • 0030752709 scopus 로고    scopus 로고
    • Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
    • DiFiglia M., Sapp E., Chase K. O., Davies S. W., Bates G. P., Vonsattel J.-P., Aronin N. A. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science. 277:1997;1990-1993.
    • (1997) Science , vol.277 , pp. 1990-1993
    • Difiglia, M.1    Sapp, E.2    Chase, K.O.3    Davies, S.W.4    Bates, G.P.5    Vonsattel, J.-P.6    Aronin, N.A.7
  • 45
    • 0031814491 scopus 로고    scopus 로고
    • Mitochondrial DNA and diseases of the nervous system: The spectrum
    • DiMauro S., Schon E. S. Mitochondrial DNA and diseases of the nervous system: The spectrum. Neuroscientist. 4:1998;53-63.
    • (1998) Neuroscientist , vol.4 , pp. 53-63
    • Dimauro, S.1    Schon, E.S.2
  • 46
    • 0029085982 scopus 로고
    • Excitatory amino acid receptors and neurodegeneration
    • Doble A. Excitatory amino acid receptors and neurodegeneration. Therapie. 50:1995;319-337.
    • (1995) Therapie , vol.50 , pp. 319-337
    • Doble, A.1
  • 49
    • 0029040355 scopus 로고
    • In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes
    • Dragunow M., Faull R. L., Lawlor P., Beilharz E. J., Singleton K., Walker E. B., Mee E. In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes. Neuroreport. 6:1995;1053-1057.
    • (1995) Neuroreport , vol.6 , pp. 1053-1057
    • Dragunow, M.1    Faull, R.L.2    Lawlor, P.3    Beilharz, E.J.4    Singleton, K.5    Walker, E.B.6    Mee, E.7
  • 51
    • 0021321899 scopus 로고
    • The natural history of Huntington's disease: Possible role of "aging genes."
    • Farrer L. A., Conneally P. M., Yu P.-L. The natural history of Huntington's disease: Possible role of "aging genes." Am. J. Hum. Genet. 18:1984;115-123.
    • (1984) Am. J. Hum. Genet. , vol.18 , pp. 115-123
    • Farrer, L.A.1    Conneally, P.M.2    Yu, P.-L.3
  • 52
    • 0026536403 scopus 로고
    • Inverse relationship between age at onset of Huntington's disease and paternal age suggests involvement of genetic imprinting
    • Farrer L. A., Cupples L. A., Kiely D. K., Conneally P. M., Myers R. H. Inverse relationship between age at onset of Huntington's disease and paternal age suggests involvement of genetic imprinting. Am. J. Hum. Genet. 50:1992;528-535.
    • (1992) Am. J. Hum. Genet. , vol.50 , pp. 528-535
    • Farrer, L.A.1    Cupples, L.A.2    Kiely, D.K.3    Conneally, P.M.4    Myers, R.H.5
  • 55
    • 0023810359 scopus 로고
    • Searching for function of tissue transglutaminase: Its possible involvement in biochemical pathway of programmed cell death
    • Fesus L., Thomazy V. Searching for function of tissue transglutaminase: Its possible involvement in biochemical pathway of programmed cell death. Adv. Expl. Biol. 231:1988;119-134.
    • (1988) Adv. Expl. Biol. , vol.231 , pp. 119-134
    • Fesus, L.1    Thomazy, V.2
  • 56
    • 0029619134 scopus 로고
    • Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration
    • Fujita K., Ando M., Yamauchi M., Nagata Y., Honda M. Alteration of transglutaminase activity in rat and human spinal cord after neuronal degeneration. Neurochem. Res. 20:1995;1195-1201.
    • (1995) Neurochem. Res. , vol.20 , pp. 1195-1201
    • Fujita, K.1    Ando, M.2    Yamauchi, M.3    Nagata, Y.4    Honda, M.5
  • 57
    • 0026648674 scopus 로고
    • Identification of programmed cell deathin situ
    • Gavrieli Y., Sherman Y., Ben-Sasson S. A. Identification of programmed cell deathin situ. J. Cell Biol. 119:1992;493-501.
    • (1992) J. Cell Biol. , vol.119 , pp. 493-501
    • Gavrieli, Y.1    Sherman, Y.2    Ben-Sasson, S.A.3
  • 58
    • 0030840881 scopus 로고    scopus 로고
    • High affinity glutamate transporters: Regulation of expression and activity
    • Gegelashvili G., Schousboe A. High affinity glutamate transporters: Regulation of expression and activity. Mol. Pharmacol. 52:1997;6-15.
    • (1997) Mol. Pharmacol. , vol.52 , pp. 6-15
    • Gegelashvili, G.1    Schousboe, A.2
  • 60
    • 0026531301 scopus 로고
    • The neostriatal mosaic: Multiple levels of compartmental organization
    • Gerfen C. R. The neostriatal mosaic: Multiple levels of compartmental organization. Trends Neurosci. 15:1992;133-139.
    • (1992) Trends Neurosci. , vol.15 , pp. 133-139
    • Gerfen, C.R.1
  • 65
    • 0028836272 scopus 로고
    • Characterization of the excitotoxic potential of the reversible succinate dehydrogenase inhibitor malonate
    • Greene J. C., Greenamyre J. T. Characterization of the excitotoxic potential of the reversible succinate dehydrogenase inhibitor malonate. J. Neurochem. 64:1995;430-436.
    • (1995) J. Neurochem. , vol.64 , pp. 430-436
    • Greene, J.C.1    Greenamyre, J.T.2
  • 70
    • 0031970977 scopus 로고    scopus 로고
    • The fatal attraction of polyglutamine-containing proteins
    • Hackam A. S., Wellington C. L., Hayden M. R. The fatal attraction of polyglutamine-containing proteins. Clin. Genet. 53:1998;233-242.
    • (1998) Clin. Genet. , vol.53 , pp. 233-242
    • Hackam, A.S.1    Wellington, C.L.2    Hayden, M.R.3
  • 71
    • 0027763445 scopus 로고
    • Cellular transglutaminases in neural development
    • Hand D., Perry M. J., Haynes L. W. Cellular transglutaminases in neural development. Int. J. Dev. Neurosci. 11:1993;709-720.
    • (1993) Int. J. Dev. Neurosci. , vol.11 , pp. 709-720
    • Hand, D.1    Perry, M.J.2    Haynes, L.W.3
  • 72
    • 0026684333 scopus 로고
    • The epidemiology of Huntington's disease
    • Harper P. S. The epidemiology of Huntington's disease. Hum. Genet. 89:1992;365-376.
    • (1992) Hum. Genet. , vol.89 , pp. 365-376
    • Harper, P.S.1
  • 73
    • 0025885733 scopus 로고
    • Neuronal loss in layer V and VI of cerebral cortex in Huntington's disease
    • Hedreen J. C., Peyser C. E., Folstein S. E., Ross C. A. Neuronal loss in layer V and VI of cerebral cortex in Huntington's disease. Neurosci. Lett. 133:1991;257-261.
    • (1991) Neurosci. Lett. , vol.133 , pp. 257-261
    • Hedreen, J.C.1    Peyser, C.E.2    Folstein, S.E.3    Ross, C.A.4
  • 74
    • 0028799793 scopus 로고
    • Early loss of neostriatal striosome neurones in Huntington's disease
    • Hedreen J. C., Folstein S. E. Early loss of neostriatal striosome neurones in Huntington's disease. J. Neuropathol. Exp. Neurol. 54:1995;105-120.
    • (1995) J. Neuropathol. Exp. Neurol. , vol.54 , pp. 105-120
    • Hedreen, J.C.1    Folstein, S.E.2
  • 78
    • 0028831934 scopus 로고
    • Quinolinic acid-induced increases in calbindin D28K immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease
    • Huang Q., Zhou D., Sapp E., Aizawa H., Ge P., Bird E. D., Vonsattel J.-P., DiFiglia M. Quinolinic acid-induced increases in calbindin D28K immunoreactivity in rat striatal neurons in vivo and in vitro mimic the pattern seen in Huntington's disease. Neuroscience. 65:1995;397-407.
    • (1995) Neuroscience , vol.65 , pp. 397-407
    • Huang, Q.1    Zhou, D.2    Sapp, E.3    Aizawa, H.4    Ge, P.5    Bird, E.D.6    Vonsattel, J.-P.7    Difiglia, M.8
  • 79
    • 0027480960 scopus 로고
    • Cell. 72:1993;971-983.
    • (1993) Cell , vol.72 , pp. 971-983
  • 80
    • 0027741301 scopus 로고
    • Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy
    • Jenkins B. G., Koroshetz W. J., Beal M. F., Rosen B. R. Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy. Neurology. 43:1993;2689-2695.
    • (1993) Neurology , vol.43 , pp. 2689-2695
    • Jenkins, B.G.1    Koroshetz, W.J.2    Beal, M.F.3    Rosen, B.R.4
  • 81
    • 0030967358 scopus 로고    scopus 로고
    • Huntington's disease: Advances in molecular and cell biology
    • Jones A. L., Wood J. D., Harper P. S. Huntington's disease: Advances in molecular and cell biology. J. Inherit. Metab. Dis. 20:1997;125-138.
    • (1997) J. Inherit. Metab. Dis. , vol.20 , pp. 125-138
    • Jones, A.L.1    Wood, J.D.2    Harper, P.S.3
  • 82
    • 0032014092 scopus 로고    scopus 로고
    • Transglutaminase action imitates Huntington's disease: Selective polymerization of Huntingtin containing expanded polyglutamine
    • Kahlem P., Green H., Dijan P. Transglutaminase action imitates Huntington's disease: Selective polymerization of Huntingtin containing expanded polyglutamine. Mol. Cell. 1:1998;595-601.
    • (1998) Mol. Cell , vol.1 , pp. 595-601
    • Kahlem, P.1    Green, H.2    Dijan, P.3
  • 85
    • 0015383455 scopus 로고
    • Apoptosis: A basic biological phenomenon with wide ranging implications in tissue kinetics
    • Kerr J. F., Wyllie A. H., Currie A. R. Apoptosis: A basic biological phenomenon with wide ranging implications in tissue kinetics. Br. J. Cancer. 26:1972;239-247.
    • (1972) Br. J. Cancer , vol.26 , pp. 239-247
    • Kerr, J.F.1    Wyllie, A.H.2    Currie, A.R.3
  • 86
    • 0022993126 scopus 로고
    • Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity
    • Koh J.-Y., Peters S., Choi D. W. Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity. Science. 234:1986;73-76.
    • (1986) Science , vol.234 , pp. 73-76
    • Koh, J.-Y.1    Peters, S.2    Choi, D.W.3
  • 87
    • 0029833062 scopus 로고    scopus 로고
    • Spinocerbellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase
    • Koshy B., Matilla T., Burright E. N., Merry D. E., Fischbeck K. H., Orr H. T., Zoghbi H. Z. Spinocerbellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase. Hum. Mol. Genet. 5:1996;1311-1318.
    • (1996) Hum. Mol. Genet. , vol.5 , pp. 1311-1318
    • Koshy, B.1    Matilla, T.2    Burright, E.N.3    Merry, D.E.4    Fischbeck, K.H.5    Orr, H.T.6    Zoghbi, H.Z.7
  • 90
    • 0027282252 scopus 로고
    • AMPA and NMDA binding sites in the hypothalamic lateral tuberal nucleus: Implications for Huntington's disease
    • Kremer B., Tallaksen-Greene S. J., Albin R. L. AMPA and NMDA binding sites in the hypothalamic lateral tuberal nucleus: Implications for Huntington's disease. Neurology. 43:1993;1593-1595.
    • (1993) Neurology , vol.43 , pp. 1593-1595
    • Kremer, B.1    Tallaksen-Greene, S.J.2    Albin, R.L.3
  • 92
    • 0026548964 scopus 로고
    • Weight loss in Huntington's disease
    • Kremer H. P. H., Roos R. A. C. Weight loss in Huntington's disease. Arch. Neurol. 49:1992;349.
    • (1992) Arch. Neurol. , vol.49 , pp. 349
    • Kremer, H.P.H.1    Roos, R.A.C.2
  • 93
    • 0025153344 scopus 로고
    • Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease
    • Kuwert T., Lange H. W., Langen K. J., Herzog H., Aulich A., Feinendegen L. E. Cortical and subcortical glucose consumption measured by PET in patients with Huntington's disease. Brain. 113:1990;1405-1423.
    • (1990) Brain , vol.113 , pp. 1405-1423
    • Kuwert, T.1    Lange, H.W.2    Langen, K.J.3    Herzog, H.4    Aulich, A.5    Feinendegen, L.E.6
  • 95
    • 0031816414 scopus 로고    scopus 로고
    • Apoptosis, excitotoxicity, and neuropathology
    • Leist M., Nicotera P. Apoptosis, excitotoxicity, and neuropathology. Exp. Cell Res. 239:1998;183-201.
    • (1998) Exp. Cell Res. , vol.239 , pp. 183-201
    • Leist, M.1    Nicotera, P.2
  • 97
    • 0031945025 scopus 로고    scopus 로고
    • Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats
    • Li S. H., Li X. J. Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats. Hum. Mol. Genet. 7:1998;777-782.
    • (1998) Hum. Mol. Genet. , vol.7 , pp. 777-782
    • Li, S.H.1    Li, X.J.2
  • 99
    • 0029899868 scopus 로고    scopus 로고
    • Huntingtin-associated protein (HAP1): Discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase
    • Li X.-J., Sharp A. H., Li S.-H., Dawson T. M., Snyder S. H., Ross C. A. Huntingtin-associated protein (HAP1): Discrete neuronal localizations in the brain resemble those of neuronal nitric oxide synthase. Proc. Natl. Acad. Sci. USA. 93:1996;4839-4844.
    • (1996) Proc. Natl. Acad. Sci. USA , vol.93 , pp. 4839-4844
    • Li, X.-J.1    Sharp, A.H.2    Li, S.-H.3    Dawson, T.M.4    Snyder, S.H.5    Ross, C.A.6
  • 101
    • 0028762647 scopus 로고
    • Excitatory amino acids as a final common pathway for neurological disorders
    • Lipton S. A., Rosenberg P. A. Excitatory amino acids as a final common pathway for neurological disorders. N. Engl. J. Med. 330:1994;613-622.
    • (1994) N. Engl. J. Med. , vol.330 , pp. 613-622
    • Lipton, S.A.1    Rosenberg, P.A.2
  • 102
    • 0026357457 scopus 로고
    • 3-Nitropropionic acid-exogenous animal toxin and possible human striatal toxin
    • Ludolph A. C., He F., Spencer P. S., Hammerstad J., Sabri M. 3-Nitropropionic acid-exogenous animal toxin and possible human striatal toxin. Can. J. Neurol. Sci. 18:1991;492-498.
    • (1991) Can. J. Neurol. Sci. , vol.18 , pp. 492-498
    • Ludolph, A.C.1    He, F.2    Spencer, P.S.3    Hammerstad, J.4    Sabri, M.5
  • 103
    • 0030273702 scopus 로고    scopus 로고
    • Huntington's disease: Translating a CAG repeat into a pathogenetic model
    • MacDonald M. E., Gusella J. F. Huntington's disease: Translating a CAG repeat into a pathogenetic model. Curr. Opin. Neurobiol. 6:1996;638-643.
    • (1996) Curr. Opin. Neurobiol. , vol.6 , pp. 638-643
    • MacDonald, M.E.1    Gusella, J.F.2
  • 104
    • 0001891547 scopus 로고    scopus 로고
    • The neurobiology of Huntington's disease
    • Cambridge: Saunders, The University Press. p. 317-358
    • MacMillan J., Quarrell O. The neurobiology of Huntington's disease. Huntington's Disease. 1996;Saunders, The University Press, Cambridge. p. 317-358.
    • (1996) Huntington's Disease
    • MacMillan, J.1    Quarrell, O.2
  • 106
    • 0031056685 scopus 로고    scopus 로고
    • Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation
    • Mangiarini L., Sathasivam K., Mahal A., Mott R., Seller M., Bates G. P. Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nature Genet. 15:1997;197-200.
    • (1997) Nature Genet. , vol.15 , pp. 197-200
    • Mangiarini, L.1    Sathasivam, K.2    Mahal, A.3    Mott, R.4    Seller, M.5    Bates, G.P.6
  • 109
    • 0017913106 scopus 로고
    • Kainate-induced degeneration of neostriatal neurons: Dependency upon corticostriatal tract
    • McGeer E. G., McGeer P. L., Singh K. Kainate-induced degeneration of neostriatal neurons: Dependency upon corticostriatal tract. Brain Res. 139:1978;381-383.
    • (1978) Brain Res. , vol.139 , pp. 381-383
    • McGeer, E.G.1    McGeer, P.L.2    Singh, K.3
  • 113
    • 0024596865 scopus 로고
    • The excitatory amino acid receptors: Their classes, pharmacology, and distinct properties in the function of the central nervous system
    • Monaghan D. T., Bridges R. J., Cotman C. W. The excitatory amino acid receptors: Their classes, pharmacology, and distinct properties in the function of the central nervous system. Annu. Rev. Pharmacol. Toxicol. 29:1989;365-402.
    • (1989) Annu. Rev. Pharmacol. Toxicol. , vol.29 , pp. 365-402
    • Monaghan, D.T.1    Bridges, R.J.2    Cotman, C.W.3
  • 114
    • 0028842309 scopus 로고
    • Unstable triplet repeat diseases
    • Monckton D. G., Caskey C. T. Unstable triplet repeat diseases. Circulation. 91:1995;513-520.
    • (1995) Circulation , vol.91 , pp. 513-520
    • Monckton, D.G.1    Caskey, C.T.2
  • 115
    • 0001810594 scopus 로고    scopus 로고
    • Psychiatric aspects of Huntington's disease
    • P. Harper. Cambridge: SaundersThe University Press
    • Morris M., Scourfield J. Psychiatric aspects of Huntington's disease. Harper P. Huntington's Disease. 1996;73-122 SaundersThe University Press, Cambridge.
    • (1996) Huntington's Disease , pp. 73-122
    • Morris, M.1    Scourfield, J.2
  • 117
    • 85068245770 scopus 로고    scopus 로고
    • Effects of alpha-phenyl-tert
    • Nakao N., Brundin P. Effects of alpha-phenyl-tert. Neuroscience. 76:1997;749-761.
    • (1997) Neuroscience , vol.76 , pp. 749-761
    • Nakao, N.1    Brundin, P.2
  • 120
    • 0030581105 scopus 로고    scopus 로고
    • In sity end-labeling detects necrosis of hippocampal pyramidal cells induced by kainic acid
    • Nishiyama K., Kwak S., Takekoshi S., Watanabe K., Kanazawa I. In sity end-labeling detects necrosis of hippocampal pyramidal cells induced by kainic acid. Neurosci. Lett. 212:1996;139-142.
    • (1996) Neurosci. Lett. , vol.212 , pp. 139-142
    • Nishiyama, K.1    Kwak, S.2    Takekoshi, S.3    Watanabe, K.4    Kanazawa, I.5
  • 122
    • 0014668762 scopus 로고
    • Brain lesion, obesity and other disturbances in mice treated with monosodium glutamate
    • Olney J. W. Brain lesion, obesity and other disturbances in mice treated with monosodium glutamate. Science. 164:1969;719-721.
    • (1969) Science , vol.164 , pp. 719-721
    • Olney, J.W.1
  • 124
    • 0029072690 scopus 로고
    • Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models
    • Portera-Cailliau C., Hedreen J. C., Price D. L., Koliatsos V. E. Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models. J. Neurosci. 15:1995;3775-3787.
    • (1995) J. Neurosci. , vol.15 , pp. 3775-3787
    • Portera-Cailliau, C.1    Hedreen, J.C.2    Price, D.L.3    Koliatsos, V.E.4
  • 125
    • 0028283985 scopus 로고
    • Glutamine repeats as polar zippers: Their possible role in inherited neurodegenerative diseases
    • Perutz M. F., Johnson T., Suzuki M., Finch J. T. Glutamine repeats as polar zippers: Their possible role in inherited neurodegenerative diseases. Proc. Natl. Acad. Sci. USA. 91:1994;5355-5358.
    • (1994) Proc. Natl. Acad. Sci. USA , vol.91 , pp. 5355-5358
    • Perutz, M.F.1    Johnson, T.2    Suzuki, M.3    Finch, J.T.4
  • 126
    • 0001899075 scopus 로고    scopus 로고
    • The clinical neurology of Huntington's disease
    • P. Harper. Cambridge: SaundersThe University Press
    • Quarrell O., Harper P. The clinical neurology of Huntington's disease. Harper P. Huntington's Disease. 1996;31-72 SaundersThe University Press, Cambridge.
    • (1996) Huntington's Disease , pp. 31-72
    • Quarrell, O.1    Harper, P.2
  • 129
    • 0030267458 scopus 로고    scopus 로고
    • Rho: Theme and variations
    • Ridley A. J. Rho: Theme and variations. Curr. Biol. 6:1996;1256-1264.
    • (1996) Curr. Biol. , vol.6 , pp. 1256-1264
    • Ridley, A.J.1
  • 130
    • 0029095105 scopus 로고
    • A mutation in glyceraldehyde-3-phosphate dehydrogenase alters endocytosis in CHO cells
    • Robbins A. R., Ward R. D., Oliver C. A mutation in glyceraldehyde-3-phosphate dehydrogenase alters endocytosis in CHO cells. J. Cell Biol. 130:1995;1093-1104.
    • (1995) J. Cell Biol. , vol.130 , pp. 1093-1104
    • Robbins, A.R.1    Ward, R.D.2    Oliver, C.3
  • 131
    • 0002885477 scopus 로고
    • Neuronal nuclear-cytoplasmic changes in Huntington's chorea: Electron microscope investigations
    • T. N. Chase, N. S. Wexler, & A. Barbeau. New York: Raven Press
    • Roizin L., Stellar S., Liu J. C. Neuronal nuclear-cytoplasmic changes in Huntington's chorea: Electron microscope investigations. Chase T. N., Wexler N. S., Barbeau A. Advances in Neurology, Vol. 23, Huntington's Disease. 1979;95-122 Raven Press, New York.
    • (1979) Advances in Neurology, Vol. 23, Huntington's Disease , pp. 95-122
    • Roizin, L.1    Stellar, S.2    Liu, J.C.3
  • 132
    • 0020566596 scopus 로고
    • Nuclear membrane indentations in Huntington's chorea
    • Ross R. A. C., Bots G. Th. A. M. Nuclear membrane indentations in Huntington's chorea. J. Neurol. Sci. 61:1983;31-47.
    • (1983) J. Neurol. Sci. , vol.61 , pp. 31-47
    • Ross, R.A.C.1    Bots, G.th.a.m.2
  • 133
    • 0029089172 scopus 로고
    • When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases
    • Ross C. A. When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases. Neuron. 15:1995;493-496.
    • (1995) Neuron , vol.15 , pp. 493-496
    • Ross, C.A.1
  • 134
    • 0031446233 scopus 로고    scopus 로고
    • Intranuclear neuronal inclusions: A common pathogenetic mechanism for glutamine-repeat neurodegenerative diseases
    • Ross C. A. Intranuclear neuronal inclusions: A common pathogenetic mechanism for glutamine-repeat neurodegenerative diseases. Neuron. 19:1997;1147-1150.
    • (1997) Neuron , vol.19 , pp. 1147-1150
    • Ross, C.A.1
  • 135
    • 0019796509 scopus 로고
    • Body weight and dietary factors on Huntington's disease patients compared with matched controls
    • Sanberg P. R., Fibiger H. C., Mark R. F. Body weight and dietary factors on Huntington's disease patients compared with matched controls. Med. J. Aust. 1:1981;407-409.
    • (1981) Med. J. Aust. , vol.1 , pp. 407-409
    • Sanberg, P.R.1    Fibiger, H.C.2    Mark, R.F.3
  • 138
    • 0028263059 scopus 로고
    • Metabotropic glutamate receptors and neuronal degenerative disorders
    • Schoepp D. D., Sacaan A. I. Metabotropic glutamate receptors and neuronal degenerative disorders. Neurobiol. Aging. 15:1994;261-263.
    • (1994) Neurobiol. Aging , vol.15 , pp. 261-263
    • Schoepp, D.D.1    Sacaan, A.I.2
  • 140
    • 0031589740 scopus 로고    scopus 로고
    • Free radicals scavengers protect dopaminergic cell lines from apoptosis by complex I inhibitors
    • Seaton T. A., Copoper J. M., Schapira A. H. Free radicals scavengers protect dopaminergic cell lines from apoptosis by complex I inhibitors. Brain Res. 777:1997;110-118.
    • (1997) Brain Res. , vol.777 , pp. 110-118
    • Seaton, T.A.1    Copoper, J.M.2    Schapira, A.H.3
  • 141
    • 0023914367 scopus 로고
    • Loss of matrix calcium-binding protein-containing neurons in Huntington's disease
    • Seto-Oshima A., Emson P. C., Lawson E., Mountjoy C. Q., Carrasco L. H. Loss of matrix calcium-binding protein-containing neurons in Huntington's disease. Lancet. 4:1988;1252-1255.
    • (1988) Lancet , vol.4 , pp. 1252-1255
    • Seto-Oshima, A.1    Emson, P.C.2    Lawson, E.3    Mountjoy, C.Q.4    Carrasco, L.H.5
  • 143
    • 0029991245 scopus 로고    scopus 로고
    • Neurobiology of Huntington's disease
    • Sharp A. H., Ross C. A. Neurobiology of Huntington's disease. Neurobiol. Dis. 3:1996;3-15.
    • (1996) Neurobiol. Dis. , vol.3 , pp. 3-15
    • Sharp, A.H.1    Ross, C.A.2
  • 144
    • 0031859745 scopus 로고    scopus 로고
    • Neuronal death in cultured murine cortical cells is induced by inhibition of GAPDH and triosephosphate isomerase
    • Sheline C. T., Choi D. W. Neuronal death in cultured murine cortical cells is induced by inhibition of GAPDH and triosephosphate isomerase. Neurobiol. Dis. 5:1998;47-54.
    • (1998) Neurobiol. Dis. , vol.5 , pp. 47-54
    • Sheline, C.T.1    Choi, D.W.2
  • 145
    • 0030580287 scopus 로고    scopus 로고
    • Why are mitochondria involved in apoptosis? Permeability transition pores and apoptosis as selective mechanisms to eliminate superoxide-producing mitochondria and cell
    • Skulachev V. P. Why are mitochondria involved in apoptosis? Permeability transition pores and apoptosis as selective mechanisms to eliminate superoxide-producing mitochondria and cell. FEBS Lett. 397:1996;7-10.
    • (1996) FEBS Lett. , vol.397 , pp. 7-10
    • Skulachev, V.P.1
  • 152
    • 0032575750 scopus 로고    scopus 로고
    • Caspases: Enemies within
    • Thornberry N. A., Lazebnik Y. Caspases: Enemies within. Science. 281:1998;1312-1316.
    • (1998) Science , vol.281 , pp. 1312-1316
    • Thornberry, N.A.1    Lazebnik, Y.2
  • 153
    • 0028234720 scopus 로고
    • Instability of CAG repeats in Huntington's disease: Relation to parental transmission and age of onset
    • Trottier Y., Biancalana V., Mandel J.-L. Instability of CAG repeats in Huntington's disease: Relation to parental transmission and age of onset. J. Med. Genet. 31:1994;377-382.
    • (1994) J. Med. Genet. , vol.31 , pp. 377-382
    • Trottier, Y.1    Biancalana, V.2    Mandel, J.-L.3
  • 155
    • 0030819255 scopus 로고    scopus 로고
    • Huntington's disease gene product, huntingtin, associates with microtubules in vitro
    • Tukamoto T., Nukina N., Ide K., Kanazawa I. Huntington's disease gene product, huntingtin, associates with microtubules in vitro. Mol. Brain Res. 51:1997;8-14.
    • (1997) Mol. Brain Res. , vol.51 , pp. 8-14
    • Tukamoto, T.1    Nukina, N.2    Ide, K.3    Kanazawa, I.4
  • 156
    • 0031867231 scopus 로고    scopus 로고
    • Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways
    • Velier J., Kim M., Schwarz C., Kim T. W., Sapp E., Chase K., Aronin N., DiFiglia M. Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways. Exp. Neurol. 152:1998;34-40.
    • (1998) Exp. Neurol. , vol.152 , pp. 34-40
    • Velier, J.1    Kim, M.2    Schwarz, C.3    Kim, T.W.4    Sapp, E.5    Chase, K.6    Aronin, N.7    Difiglia, M.8
  • 158
    • 0031747231 scopus 로고    scopus 로고
    • Genetic polymorphisms adjacent to the CAG repeat influence clinical features at onset in Huntington's disease
    • Vuillaume I., Vermersch P., Destee A., Petit H., Salbonniere B. Genetic polymorphisms adjacent to the CAG repeat influence clinical features at onset in Huntington's disease. J. Neurol. Neurosurg. Psychiatry. 64:1998;758-762.
    • (1998) J. Neurol. Neurosurg. Psychiatry , vol.64 , pp. 758-762
    • Vuillaume, I.1    Vermersch, P.2    Destee, A.3    Petit, H.4    Salbonniere, B.5
  • 161
    • 0029895292 scopus 로고    scopus 로고
    • Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease
    • Weeks R. A., Piccini P., Harding A. E., Brooks D. J. Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease. Ann. Neurol. 40:1996;49-54.
    • (1996) Ann. Neurol. , vol.40 , pp. 49-54
    • Weeks, R.A.1    Piccini, P.2    Harding, A.E.3    Brooks, D.J.4
  • 162
  • 164
    • 0001918396 scopus 로고
    • Cell death: A new classification separating apoptosis from necrosis
    • I. D. Bowen, & R. A. Lockshin. New York: Chapman and Hall
    • Wyllie A. H. Cell death: A new classification separating apoptosis from necrosis. Bowen I. D., Lockshin R. A. Cell Death in Biology and Pathology. 1981;9-34 Chapman and Hall, New York.
    • (1981) Cell Death in Biology and Pathology , pp. 9-34
    • Wyllie, A.H.1
  • 167
    • 0030848374 scopus 로고    scopus 로고
    • Mitochondrial implication in accidental and programmed cell death: Apoptosis and necrosis
    • Zamzami N., Hirsch T., Dallaporta B., Petit P. X., Kroemer G. Mitochondrial implication in accidental and programmed cell death: Apoptosis and necrosis. J. Bionerg. Biomembr. 29:1997;185-193.
    • (1997) J. Bionerg. Biomembr. , vol.29 , pp. 185-193
    • Zamzami, N.1    Hirsch, T.2    Dallaporta, B.3    Petit, P.X.4    Kroemer, G.5
  • 168
    • 84993912315 scopus 로고
    • Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
    • Zeitlin S., Liu J.-P., Chapman D. L., Papaioannou V. E., Efstratiadis A. Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue. Nature Genet. 11:1995;155-163.
    • (1995) Nature Genet. , vol.11 , pp. 155-163
    • Zeitlin, S.1    Liu, J.-P.2    Chapman, D.L.3    Papaioannou, V.E.4    Efstratiadis, A.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.