메뉴 건너뛰기




Volumn 2012, Issue , 2012, Pages

Nucleic acid-based therapy approaches for Huntington's disease

Author keywords

[No Author keywords available]

Indexed keywords


EID: 84866156940     PISSN: 20901852     EISSN: 20901860     Source Type: Journal    
DOI: 10.1155/2012/358370     Document Type: Review
Times cited : (7)

References (115)
  • 1
    • 0022876328 scopus 로고
    • Huntington's disease: Pathogenesis and management
    • Martin J. B., Gusella J. F., Huntington's disease: pathogenesis and management. The New England Journal of Medicine 1986 315 20 1267 1276 2-s2.0-0022876328 (Pubitemid 17049686)
    • (1986) New England Journal of Medicine , vol.315 , Issue.20 , pp. 1267-1276
    • Martin, J.B.1    Gusella, J.F.2
  • 2
    • 0027480960 scopus 로고
    • A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group
    • A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell 1993 72 6 971 983
    • (1993) Cell , vol.72 , Issue.6 , pp. 971-983
  • 6
    • 0035805504 scopus 로고    scopus 로고
    • Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing
    • 2-s2.0-0035805504 10.1074/jbc.C100044200
    • Rigamonti D., Sipione S., Goffredo D., Zuccato C., Fossale E., Cattaneo E., Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing. The Journal of Biological Chemistry 2001 276 18 14545 14548 2-s2.0-0035805504 10.1074/jbc.C100044200
    • (2001) The Journal of Biological Chemistry , vol.276 , Issue.18 , pp. 14545-14548
    • Rigamonti, D.1    Sipione, S.2    Goffredo, D.3    Zuccato, C.4    Fossale, E.5    Cattaneo, E.6
  • 9
    • 0030878098 scopus 로고    scopus 로고
    • Fast transport and retrograde movement of huntingtin and HAP 1 in axons
    • Block-Galarza J., Chase K. O., Sapp E., Vaughn K. T., Valle R. B., DiFiglia M., Aronin N., Fast transport and retrograde movement of huntingtin and HAP 1 in axons. NeuroReport 1997 8 9-10 2247 2251 2-s2.0-0030878098 (Pubitemid 27319681)
    • (1997) NeuroReport , vol.8 , Issue.9-10 , pp. 2247-2251
    • Block-Galarza, J.1    Chase, K.O.2    Sapp, E.3    Vaughn, K.T.4    Valle, R.B.5    DiFiglia, M.6    Aronin, N.7
  • 10
    • 3142636768 scopus 로고    scopus 로고
    • Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
    • DOI 10.1016/j.cell.2004.06.018, PII S0092867404006191
    • Gauthier L. R., Charrin B. C., Borrell-Pagès M., Dompierre J. P., Rangone H., Cordelières F. P., De Mey J., MacDonald M. E., Lemann V., Humbert S., Saudou F., Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell 2004 118 1 127 138 2-s2.0-3142636768 10.1016/j.cell.2004.06.018 (Pubitemid 38902819)
    • (2004) Cell , vol.118 , Issue.1 , pp. 127-138
    • Gauthier, L.R.1    Charrin, B.C.2    Borrell-Pages, M.3    Dompierre, J.P.4    Rangone, H.5    Cordelieres, F.P.6    De Mey, J.7    MacDonald, M.E.8    Lessmann, V.9    Humbert, S.10    Saudou, F.11
  • 14
    • 0035816627 scopus 로고    scopus 로고
    • Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95
    • 2-s2.0-0035816627 10.1074/jbc.M103501200
    • Sun Y., Savanenin A., Reddy P. H., Liu Y. F., Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. The Journal of Biological Chemistry 2001 276 27 24713 24718 2-s2.0-0035816627 10.1074/jbc.M103501200
    • (2001) The Journal of Biological Chemistry , vol.276 , Issue.27 , pp. 24713-24718
    • Sun, Y.1    Savanenin, A.2    Reddy, P.H.3    Liu, Y.F.4
  • 15
    • 24344461460 scopus 로고    scopus 로고
    • Synaptic dysfunction in Huntington's disease: A new perspective
    • DOI 10.1007/s00018-005-5084-5
    • Smith R., Brundin P., Li J. Y., Synaptic dysfunction in Huntington's disease: a new perspective. Cellular and Molecular Life Sciences 2005 62 17 1901 1912 2-s2.0-24344461460 10.1007/s00018-005-5084-5 (Pubitemid 41261838)
    • (2005) Cellular and Molecular Life Sciences , vol.62 , Issue.17 , pp. 1901-1912
    • Smith, R.1    Brundin, P.2    Li, J.-Y.3
  • 16
    • 0017090806 scopus 로고
    • Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids
    • 2-s2.0-0017090806
    • McGeer E. G., McGeer P. L., Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids. Nature 1976 263 5577 517 519 2-s2.0-0017090806
    • (1976) Nature , vol.263 , Issue.5577 , pp. 517-519
    • McGeer, E.G.1    McGeer, P.L.2
  • 17
    • 0022356170 scopus 로고
    • Excitotoxin lesions do not mimic the alteration of somatostatin in Huntington's disease
    • DOI 10.1016/0006-8993(85)91283-1
    • Beal M. F., Marshall P. E., Burd G. D., Excitotoxin lesions do not mimic the alteration of somatostatin in Huntington's disease. Brain Research 1985 361 1-2 135 145 2-s2.0-0022356170 (Pubitemid 16185394)
    • (1985) Brain Research , vol.361 , Issue.1-2 , pp. 135-145
    • Beal, M.F.1    Marshall, P.E.2    Burd, G.D.3
  • 18
    • 0022446150 scopus 로고
    • Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid
    • Beal M. F., Kowall N. W., Ellison D. W., Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature 1986 321 6066 168 171 2-s2.0-0022446150 (Pubitemid 16022019)
    • (1986) Nature , vol.321 , Issue.6066 , pp. 168-171
    • Beal, M.F.1    Kowall, N.W.2    Ellison, D.W.3
  • 19
    • 0027730251 scopus 로고
    • Intrastriatal injections of quinolinic acid or kainic acid: Differential patterns of cell survival and the effects of data analysis on outcome
    • DOI 10.1006/exnr.1993.1197
    • Roberts R. C., Ahn A., Swartz K. J., Beal M. F., DiFiglia M., Intrastriatal injections of quinolinic acid or kainic acid: differential patterns of cell survival and the effects of data analysis on outcome. Experimental Neurology 1993 124 2 274 282 2-s2.0-0027730251 10.1006/exnr.1993. 1197 (Pubitemid 24041774)
    • (1993) Experimental Neurology , vol.124 , Issue.2 , pp. 274-282
    • Roberts, R.C.1    Ahn, A.2    Swartz, K.J.3    Beal, M.F.4    DiFiglia, M.5
  • 20
    • 0025279038 scopus 로고
    • A primate model of Huntington's disease: Behavioral and anatomical studies of unilateral excitotoxic lesions of the caudate-putamen in the baboon
    • DOI 10.1016/0014-4886(90)90014-J
    • Hantraye P., Riche D., Maziere M., Isacson O., A primate model of Huntington's disease: behavioral and anatomical studies of unilateral excitotoxic lesions of the caudate-putamen in the baboon. Experimental Neurology 1990 108 2 91 104 2-s2.0-0025279038 10.1016/0014-4886(90)90014-J (Pubitemid 20153928)
    • (1990) Experimental Neurology , vol.108 , Issue.2 , pp. 91-104
    • Hantraye, P.1    Riche, D.2    Maziere, M.3    Isacson, O.4
  • 21
    • 0028862452 scopus 로고
    • Behavioral pathology induced by repeated systemic injections of 3-nitropropionic acid mimics the motoric symptoms of Huntington's disease
    • 2-s2.0-0028862452 10.1016/0006-8993(95)00901-2
    • Borlongan C. V., Koutouzis T. K., Freeman T. B., Cahill D. W., Sanberg P. R., Behavioral pathology induced by repeated systemic injections of 3-nitropropionic acid mimics the motoric symptoms of Huntington's disease. Brain Research 1995 697 1-2 254 257 2-s2.0-0028862452 10.1016/0006-8993(95)00901-2
    • (1995) Brain Research , vol.697 , Issue.1-2 , pp. 254-257
    • Borlongan, C.V.1    Koutouzis, T.K.2    Freeman, T.B.3    Cahill, D.W.4    Sanberg, P.R.5
  • 22
    • 0030997707 scopus 로고    scopus 로고
    • Quantifiable bradykinesia, gait abnormalities and Huntington's disease- like striatal lesions in rats chronically treated with 3-nitropropionic acid
    • DOI 10.1016/S0306-4522(96)00602-1, PII S0306452296006021
    • Guyot M. C., Hantraye P., Dolan R., Palfi S., Maziére M., Brouillet E., Quantifiable bradykinesia, gait abnormalities and Huntington's disease- like striatal lesions in rats chronically treated with 3-nitropropionic acid. Neuroscience 1997 79 1 45 56 2-s2.0-0030997707 10.1016/S0306-4522(96) 00602-1 (Pubitemid 27244999)
    • (1997) Neuroscience , vol.79 , Issue.1 , pp. 45-56
    • Guyot, M.-C.1    Hantraye, P.2    Dolan, R.3    Palfi, S.4    Maziere, M.5    Brouillet, E.6
  • 23
    • 16044373842 scopus 로고    scopus 로고
    • Exon I of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
    • DOI 10.1016/S0092-8674(00)81369-0
    • Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., Lawton M., Trottier Y., Lehrach H., Davies S. W., Bates G. P., Exon I of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996 87 3 493 506 2-s2.0-16044373842 10.1016/S0092-8674(00)81369-0 (Pubitemid 26374323)
    • (1996) Cell , vol.87 , Issue.3 , pp. 493-506
    • Mangiarini, L.1    Sathasivam, K.2    Seller, M.3    Cozens, B.4    Harper, A.5    Hetherington, C.6    Lawton, M.7    Trottier, Y.8    Lehrach, H.9    Davies, S.W.10    Bates, G.P.11
  • 27
    • 0037107191 scopus 로고    scopus 로고
    • Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice
    • Menalled L. B., Sison J. D., Wu Y., Olivieri M., Li X. J., Li H., Zeitlin S., Chesselet M. F., Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice. Journal of Neuroscience 2002 22 18 8266 8276 2-s2.0-0037107191 (Pubitemid 35379132)
    • (2002) Journal of Neuroscience , vol.22 , Issue.18 , pp. 8266-8276
    • Menalled, L.B.1    Sison, J.D.2    Wu, Y.3    Olivieri, M.4    Li, X.-J.5    Li, H.6    Zeitlin, S.7    Chesselet, M.-F.8
  • 28
    • 0034057350 scopus 로고    scopus 로고
    • Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome
    • DOI 10.1136/jnnp.68.2.238
    • Reuter I., Hu M. T. M., Andrews T. C., Brooks D. J., Clough C., Ray Chaudhuri K., Late onset levodopa responsive Huntington's disease with minimal chorea masquerading as Parkinson plus syndrome. Journal of Neurology Neurosurgery and Psychiatry 2000 68 2 238 241 2-s2.0-0034057350 10.1136/jnnp.68.2.238 (Pubitemid 30245854)
    • (2000) Journal of Neurology Neurosurgery and Psychiatry , vol.68 , Issue.2 , pp. 238-241
    • Reuter, I.1    Hu, M.T.M.2    Andrews, T.C.3    Brooks, D.J.4    Clough, C.5    Ray Chaudhuri, K.6
  • 29
    • 0035191368 scopus 로고    scopus 로고
    • Successful multimodality treatment of severe behavioral disturbance in a patient with advanced Huntington's disease
    • DOI 10.1176/appi.ajp.158.12.1966
    • Blass D. M., Steinberg M., Leroi I., Lyketsos C. G., Successful multimodality treatment of severe behavioral disturbance in a patient with advanced Huntington's disease. American Journal of Psychiatry 2001 158 12 1966 1972 2-s2.0-0035191368 10.1176/appi.ajp.158.12.1966 (Pubitemid 33104100)
    • (2001) American Journal of Psychiatry , vol.158 , Issue.12 , pp. 1966-1972
    • Blass, D.M.1    Steinberg, M.2    Leroi, I.3    Lyketsos, C.G.4
  • 30
    • 0036869757 scopus 로고    scopus 로고
    • Tetrabenazine treatment for Huntington's disease-associated chorea
    • DOI 10.1097/00002826-200211000-00003
    • Ondo W. G., Tintner R., Thomas M., Jankovic J., Tetrabenazine treatment for Huntington's disease-associated chorea. Clinical Neuropharmacology 2002 25 6 300 302 2-s2.0-0036869757 10.1097/00002826-200211000-00003 (Pubitemid 36025267)
    • (2002) Clinical Neuropharmacology , vol.25 , Issue.6 , pp. 300-302
    • Ondo, W.G.1    Tintner, R.2    Thomas, M.3    Jankovic, J.4
  • 33
    • 85009226418 scopus 로고    scopus 로고
    • 10 and remacemide in Huntington's disease
    • Kieburtz K., A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001 57 3 397 404 2-s2.0-0035859843 (Pubitemid 32757583)
    • (2001) Neurology , vol.57 , Issue.3 , pp. 397-404
    • Kieburtz, K.1
  • 34
    • 0345600893 scopus 로고    scopus 로고
    • Dosage effects of riluzole in Huntington's disease: A multicenter placebo-controlled study
    • Marshall F. J., Dosage effects of riluzole in Huntington's disease: a multicenter placebo-controlled study. Neurology 2003 61 11 1551 1556 2-s2.0-0345600893 (Pubitemid 37505578)
    • (2003) Neurology , vol.61 , Issue.11 , pp. 1551-1556
    • Marshall, F.J.1
  • 35
    • 0037677341 scopus 로고    scopus 로고
    • Creatine therapy for Huntington's disease: Clinical and MRS findings in a 1-year pilot study
    • Tabrizi S. J., Blamire A. M., Manners D. N., Rajagopalan B., Styles P., Schapira A. H. V., Warner T. T., Creatine therapy for Huntington's disease: clinical and MRS findings in a 1-year pilot study. Neurology 2003 61 1 141 142 2-s2.0-0037677341 (Pubitemid 36818771)
    • (2003) Neurology , vol.61 , Issue.1 , pp. 141-142
    • Tabrizi, S.J.1    Blamire, A.M.2    Manners, D.N.3    Rajagopalan, B.4    Styles, P.5    Schapira, A.H.V.6    Warner, T.T.7
  • 36
    • 4444233214 scopus 로고    scopus 로고
    • Minocycline in Huntington's disease: A pilot study
    • DOI 10.1002/mds.20018
    • Thomas M., Ashizawa T., Jankovic J., Minocycline in Huntington's disease: a pilot study. Movement Disorders 2004 19 6 692 695 2-s2.0-4444233214 10.1002/mds.20018 (Pubitemid 39173025)
    • (2004) Movement Disorders , vol.19 , Issue.6 , pp. 692-695
    • Thomas, M.1    Ashizawa, T.2    Jankovic, J.3
  • 38
    • 34948855498 scopus 로고    scopus 로고
    • Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease
    • DOI 10.1172/JCI31778
    • Cho S. R., Benraiss A., Chmielnicki E., Samdani A., Economides A., Goldman S. A., Induction of neostriatal neurogenesis slows disease progression in a transgenic murine model of Huntington disease. Journal of Clinical Investigation 2007 117 10 2889 2902 2-s2.0-34948855498 10.1172/JCI31778 (Pubitemid 47529619)
    • (2007) Journal of Clinical Investigation , vol.117 , Issue.10 , pp. 2889-2902
    • Cho, S.-R.1    Benraiss, A.2    Chmielnicki, E.3    Samdani, A.4    Economides, A.5    Goldman, S.A.6
  • 40
    • 0028574874 scopus 로고
    • Implantation of polymer-encapsulated human nerve growth factor-secreting fibroblasts attenuates the behavioral and neuropathological consequences of quinolinic acid injections into rodent striatum
    • 2-s2.0-0028574874 10.1006/exnr.1994.1193
    • Emerich D. F., Hammang J. P., Baetge E. E., Winn S. R., Implantation of polymer-encapsulated human nerve growth factor-secreting fibroblasts attenuates the behavioral and neuropathological consequences of quinolinic acid injections into rodent striatum. Experimental Neurology 1994 130 1 141 150 2-s2.0-0028574874 10.1006/exnr.1994.1193
    • (1994) Experimental Neurology , vol.130 , Issue.1 , pp. 141-150
    • Emerich, D.F.1    Hammang, J.P.2    Baetge, E.E.3    Winn, S.R.4
  • 41
    • 0029905329 scopus 로고    scopus 로고
    • Protection of the neostriatum against excitotoxic damage by neurotrophin-producing, genetically modified neural stem cells
    • Martínez-Serrano A., Björklund A., Protection of the neostriatum against excitotoxic damage by neurotrophin-producing, genetically modified neural stem cells. Journal of Neuroscience 1996 16 15 4604 4616 2-s2.0-0029905329 (Pubitemid 26251057)
    • (1996) Journal of Neuroscience , vol.16 , Issue.15 , pp. 4604-4616
    • Martinez-Serrano, A.1    Bjorklund, A.2
  • 43
    • 0028558577 scopus 로고
    • Intravenous administration of a transferrin receptor antibody-nerve growth factor conjugate prevents the degeneration of cholinergic striatal neurons in a model of Huntington disease
    • 2-s2.0-0028558577 10.1073/pnas.91.19.9077
    • Kordower J. H., Charles V., Bayer R., Bartus R. T., Putney S., Walus L. R., Friden P. M., Intravenous administration of a transferrin receptor antibody-nerve growth factor conjugate prevents the degeneration of cholinergic striatal neurons in a model of Huntington disease. Proceedings of the National Academy of Sciences of the United States of America 1994 91 19 9077 9080 2-s2.0-0028558577 10.1073/pnas.91.19.9077
    • (1994) Proceedings of the National Academy of Sciences of the United States of America , vol.91 , Issue.19 , pp. 9077-9080
    • Kordower, J.H.1    Charles, V.2    Bayer, R.3    Bartus, R.T.4    Putney, S.5    Walus, L.R.6    Friden, P.M.7
  • 44
    • 0028362474 scopus 로고
    • Intrastriatal infusion of nerve growth factor after quinolinic acid prevents reduction of cellular expression of choline acetyltransferase messenger RNA and trkA messenger RNA, but not glutamate decarboxylase messenger RNA
    • 2-s2.0-0028362474 10.1016/0306-4522(94)90229-1
    • Venero J. L., Beck K. D., Hefti F., Intrastriatal infusion of nerve growth factor after quinolinic acid prevents reduction of cellular expression of choline acetyltransferase messenger RNA and trkA messenger RNA, but not glutamate decarboxylase messenger RNA. Neuroscience 1994 61 2 257 268 2-s2.0-0028362474 10.1016/0306-4522(94)90229-1
    • (1994) Neuroscience , vol.61 , Issue.2 , pp. 257-268
    • Venero, J.L.1    Beck, K.D.2    Hefti, F.3
  • 45
    • 34147166160 scopus 로고    scopus 로고
    • Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease
    • DOI 10.1016/j.nbd.2007.01.003, PII S0969996107000216
    • Ramaswamy S., McBride J. L., Herzog C. D., Brandon E., Gasmi M., Bartus R. T., Kordower J. H., Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease. Neurobiology of Disease 2007 26 2 375 384 2-s2.0-34147166160 10.1016/j.nbd.2007.01.003 (Pubitemid 46561340)
    • (2007) Neurobiology of Disease , vol.26 , Issue.2 , pp. 375-384
    • Ramaswamy, S.1    McBride, J.L.2    Herzog, C.D.3    Brandon, E.4    Gasmi, M.5    Bartus, R.T.6    Kordower, J.H.7
  • 46
    • 77953701829 scopus 로고    scopus 로고
    • Ex vivo delivery of GDNF maintains motor function and prevents neuronal loss in a transgenic mouse model of Huntington's disease
    • 2-s2.0-77953701829 10.1016/j.expneurol.2010.03.005
    • Ebert A. D., Barber A. E., Heins B. M., Svendsen C. N., Ex vivo delivery of GDNF maintains motor function and prevents neuronal loss in a transgenic mouse model of Huntington's disease. Experimental Neurology 2010 224 1 155 162 2-s2.0-77953701829 10.1016/j.expneurol.2010.03.005
    • (2010) Experimental Neurology , vol.224 , Issue.1 , pp. 155-162
    • Ebert, A.D.1    Barber, A.E.2    Heins, B.M.3    Svendsen, C.N.4
  • 47
    • 16244373680 scopus 로고    scopus 로고
    • Lentiviral gene delivery of GDNF into the striatum of R6/2 Huntington mice fails to attenuate behavioral and neuropathological changes
    • DOI 10.1016/j.expneurol.2004.12.009
    • Popovic N., Maingay M., Kirik D., Brundin P., Lentiviral gene delivery of GDNF into the striatum of R6/2 Huntington mice fails to attenuate behavioral and neuropathological changes. Experimental Neurology 2005 193 1 65 74 2-s2.0-16244373680 10.1016/j.expneurol.2004.12.009 (Pubitemid 40463268)
    • (2005) Experimental Neurology , vol.193 , Issue.1 , pp. 65-74
    • Popovic, N.1    Maingay, M.2    Kirik, D.3    Brundin, P.4
  • 48
    • 0346993604 scopus 로고    scopus 로고
    • Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice
    • DOI 10.1016/j.expneurol.2003.09.002
    • Zala D., Bensadoun J. C., De Almeida L. P., Leavitt B. R., Gutekunst C. A., Aebischer P., Hayden M. R., Déglon N., Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice. Experimental Neurology 2004 185 1 26 35 2-s2.0-0346993604 10.1016/j.expneurol.2003.09.002 (Pubitemid 38010276)
    • (2004) Experimental Neurology , vol.185 , Issue.1 , pp. 26-35
    • Zala, D.1    Bensadoun, J.-C.2    De Almeida, L.P.3    Leavitt, B.R.4    Gutekunst, C.-A.5    Aebischer, P.6    Hayden, M.R.7    Deglon, N.8
  • 51
    • 0034979217 scopus 로고    scopus 로고
    • Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease
    • DOI 10.1006/nbdi.2001.0388
    • de Almeida L. P., Zala D., Aebischer P., Déglon N., Neuroprotective effect of a CNTF-expressing lentiviral vector in the quinolinic acid rat model of Huntington's disease. Neurobiology of Disease 2001 8 3 433 446 2-s2.0-0034979217 10.1006/nbdi.2001.0388 (Pubitemid 32566134)
    • (2001) Neurobiology of Disease , vol.8 , Issue.3 , pp. 433-446
    • De Almeida, L.P.1    Zala, D.2    Aebischer, P.3    Deglon, N.4
  • 52
    • 0036615771 scopus 로고    scopus 로고
    • Corticostriatopallidal Neuroprotection by Adenovirus-Mediated Ciliary Neurotrophic Factor Gene Transfer in a Rat Model of Progressive Striatal Degeneration
    • Mittoux V., Ouary S., Monville C., Lisovoski F., Poyot T., Condé F., Escartin C., Robichon R., Brouillet E., Peschanski M., Hantraye P., Corticostriatopallidal neuroprotection by adenovirus-mediated ciliary neurotrophic factor gene transfer in a rat model of progressive striatal degeneration. Journal of Neuroscience 2002 22 11 4478 4486 2-s2.0-0036615771 (Pubitemid 37465664)
    • (2002) Journal of Neuroscience , vol.22 , Issue.11 , pp. 4478-4486
    • Mittoux, V.1    Ouary, S.2    Monville, C.3    Lisovoski, F.4    Poyot, T.5    Conde, F.6    Escartin, C.7    Robichon, R.8    Brouillet, E.9    Peschanski, M.10    Hantraye, P.11
  • 54
    • 34247402598 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor restores synaptic plasticity in a knock-in mouse model of Huntington's disease
    • DOI 10.1523/JNEUROSCI.5113-06.2007
    • Lynch G., Kramar E. A., Rex C. S., Jia Y., Chappas D., Gall C. M., Simmons D. A., Brain-derived neurotrophic factor restores synaptic plasticity in a knock-in mouse model of Huntington's disease. Journal of Neuroscience 2007 27 16 4424 4434 2-s2.0-34247402598 10.1523/JNEUROSCI.5113-06.2007 (Pubitemid 46640726)
    • (2007) Journal of Neuroscience , vol.27 , Issue.16 , pp. 4424-4434
    • Lynch, G.1    Kramar, E.A.2    Rex, C.S.3    Jia, Y.4    Chappas, D.5    Gall, C.M.6    Simmons, D.A.7
  • 55
    • 4444350918 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease
    • DOI 10.1523/JNEUROSCI.1197-04.2004
    • Canals J. M., Pineda J. R., Torres-Peraza J. F., Bosch M., Martín-Ibañez R., Muñoz M. T., Mengod G., Ernfors P., Alberch J., Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease. Journal of Neuroscience 2004 24 35 7727 7739 2-s2.0-4444350918 10.1523/JNEUROSCI.1197-04.2004 (Pubitemid 39186828)
    • (2004) Journal of Neuroscience , vol.24 , Issue.35 , pp. 7727-7739
    • Canals, J.M.1    Pineda, J.R.2    Torres-Peraza, J.F.3    Bosch, M.4    Martin-Ibanez, R.5    Munoz, M.T.6    Mengod, G.7    Ernfors, P.8    Alberch, J.9
  • 56
    • 60349130539 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor modulates the severity of cognitive alterations induced by mutant huntingtin: Involvement of phospholipaseC γ activity and glutamate receptor expression
    • 2-s2.0-60349130539 10.1016/j.neuroscience.2008.11.024
    • Giralt A., Rodrigo T., Martín E. D., Gonzalez J. R., Milà M., Ceña V., Dierssen M., Canals J. M., Alberch J., Brain-derived neurotrophic factor modulates the severity of cognitive alterations induced by mutant huntingtin: involvement of phospholipaseC γ activity and glutamate receptor expression. Neuroscience 2009 158 4 1234 1250 2-s2.0-60349130539 10.1016/j.neuroscience.2008.11.024
    • (2009) Neuroscience , vol.158 , Issue.4 , pp. 1234-1250
    • Giralt, A.1    Rodrigo, T.2    Martín, E.D.3    Gonzalez, J.R.4    Milà, M.5    Ceña, V.6    Dierssen, M.7    Canals, J.M.8    Alberch, J.9
  • 57
    • 0034595722 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor in Huntington disease
    • DOI 10.1016/S0006-8993(00)02237-X, PII S000689930002237X
    • Ferrer I., Goutan E., Marín C., Rey M. J., Ribalta T., Brain-derived neurotrophic factor in Huntington disease. Brain Research 2000 866 1-2 257 261 2-s2.0-0034595722 10.1016/S0006-8993(00)02237-X (Pubitemid 30313028)
    • (2000) Brain Research , vol.866 , Issue.1-2 , pp. 257-261
    • Ferrer, I.1    Goutan, E.2    Marin, C.3    Rey, M.J.4    Ribalta, T.5
  • 58
    • 28644433087 scopus 로고    scopus 로고
    • Normal huntingtin function: An alternative approach to Huntington's disease
    • DOI 10.1038/nrn1806
    • Cattaneo E., Zuccato C., Tartari M., Normal huntingtin function: an alternative approach to Huntington's disease. Nature Reviews Neuroscience 2005 6 12 919 930 2-s2.0-28644433087 10.1038/nrn1806 (Pubitemid 41753086)
    • (2005) Nature Reviews Neuroscience , vol.6 , Issue.12 , pp. 919-930
    • Cattaneo, E.1    Zuccato, C.2    Tartari, M.3
  • 59
    • 0033544840 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor-mediated protection of striatal neurons in an excitotoxic rat model of Huntington's disease, as demonstrated by adenoviral gene transfer
    • 2-s2.0-0033544840 10.1089/10430349950016393
    • Bemelmans A. P., Horellou P., Pradier L., Brunet I., Colin P., Mallet J., Brain-derived neurotrophic factor-mediated protection of striatal neurons in an excitotoxic rat model of Huntington's disease, as demonstrated by adenoviral gene transfer. Human Gene Therapy 1999 10 18 2987 2997 2-s2.0-0033544840 10.1089/10430349950016393
    • (1999) Human Gene Therapy , vol.10 , Issue.18 , pp. 2987-2997
    • Bemelmans, A.P.1    Horellou, P.2    Pradier, L.3    Brunet, I.4    Colin, P.5    Mallet, J.6
  • 60
    • 2442702838 scopus 로고    scopus 로고
    • AAV-mediated gene delivery of BDNF or GDNF is neuroprotective in a model of Huntington disease
    • DOI 10.1016/j.ymthe.2004.02.016
    • Kells A. P., Fong D. M., Dragunow M., During M. J., Young D., Connor B., AAV-mediated gene delivery of BDNF or GDNF is neuroprotective in a model of Huntington disease. Molecular Therapy 2004 9 5 682 688 2-s2.0-2442702838 10.1016/j.ymthe.2004.02.016 (Pubitemid 38660421)
    • (2004) Molecular Therapy , vol.9 , Issue.5 , pp. 682-688
    • Kells, A.P.1    Fong, D.M.2    Dragunow, M.3    During, M.J.4    Young, D.5    Connor, B.6
  • 61
    • 45749153812 scopus 로고    scopus 로고
    • AAV-BDNF mediated attenuation of quinolinic acid-induced neuropathology and motor function impairment
    • DOI 10.1038/gt.2008.23, PII GT200823
    • Kells A. P., Henry R. A., Connor B., AAV-BDNF mediated attenuation of quinolinic acid-induced neuropathology and motor function impairment. Gene Therapy 2008 15 13 966 977 2-s2.0-45749153812 10.1038/gt.2008.23 (Pubitemid 351865753)
    • (2008) Gene Therapy , vol.15 , Issue.13 , pp. 966-977
    • Kells, A.P.1    Henry, R.A.2    Connor, B.3
  • 62
    • 0027468487 scopus 로고
    • Effects of biologically delivered NGF, BDNF and bFGF on striatal excitotoxic lesions
    • Frim D. M., Uhler T. A., Short M. P., Ezzedine Z. D., Klagsbrun M., Breakefield X. O., Isacson O., Effects of biologically delivered NGF, BDNF and bFGF on striatal excitotoxic lesions. NeuroReport 1993 4 4 367 370 2-s2.0-0027468487 (Pubitemid 23116724)
    • (1993) NeuroReport , vol.4 , Issue.4 , pp. 367-370
    • Frim, D.M.1    Uhler, T.A.2    Short, M.P.3    Ezzedine, Z.D.4    Klagsbrun, M.5    Breakefield, X.O.6    Isacson, O.7
  • 63
    • 49349113732 scopus 로고    scopus 로고
    • Protective effect of BDNF against beta-amyloid induced neurotoxicity in vitro and in vivo in rats
    • 2-s2.0-49349113732 10.1016/j.nbd.2008.05.012
    • Arancibia S., Silhol M., Moulière F., Meffre J., Höllinger I., Maurice T., Tapia-Arancibia L., Protective effect of BDNF against beta-amyloid induced neurotoxicity in vitro and in vivo in rats. Neurobiology of Disease 2008 31 3 316 326 2-s2.0-49349113732 10.1016/j.nbd.2008.05.012
    • (2008) Neurobiology of Disease , vol.31 , Issue.3 , pp. 316-326
    • Arancibia, S.1    Silhol, M.2    Moulière, F.3    Meffre, J.4    Höllinger, I.5    Maurice, T.6    Tapia-Arancibia, L.7
  • 64
    • 65249108107 scopus 로고    scopus 로고
    • Preventing and exploiting the oncogenic potential of integrating gene vectors
    • 2-s2.0-65249108107 10.1172/JCI38831
    • Modlich U., Baum C., Preventing and exploiting the oncogenic potential of integrating gene vectors. The Journal of Clinical Investigation 2009 119 4 755 758 2-s2.0-65249108107 10.1172/JCI38831
    • (2009) The Journal of Clinical Investigation , vol.119 , Issue.4 , pp. 755-758
    • Modlich, U.1    Baum, C.2
  • 65
    • 32944470807 scopus 로고    scopus 로고
    • Recombinant adeno-associated viral vectors as therapeutic agents to treat neurological disorders
    • DOI 10.1016/j.ymthe.2005.11.009, PII S1525001605017028
    • Mandel R. J., Manfredsson F. P., Foust K. D., Rising A., Reimsnider S., Nash K., Burger C., Recombinant adeno-associated viral vectors as therapeutic agents to treat neurological disorders. Molecular Therapy 2006 13 3 463 483 2-s2.0-32944470807 10.1016/j.ymthe.2005.11.009 (Pubitemid 43257615)
    • (2006) Molecular Therapy , vol.13 , Issue.3 , pp. 463-483
    • Mandel, R.J.1    Manfredsson, F.P.2    Foust, K.D.3    Rising, A.4    Reimsnider, S.5    Nash, K.6    Burger, C.7
  • 66
    • 77953722282 scopus 로고    scopus 로고
    • Development of gene therapy for neurological disorders
    • 2-s2.0-77953722282
    • Manfredsson F. P., Mandel R. J., Development of gene therapy for neurological disorders. Discovery Medicine 2010 9 46 204 211 2-s2.0-77953722282
    • (2010) Discovery Medicine , vol.9 , Issue.46 , pp. 204-211
    • Manfredsson, F.P.1    Mandel, R.J.2
  • 67
    • 0034737299 scopus 로고    scopus 로고
    • Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
    • 2-s2.0-0034737299
    • Yamamoto A., Lucas J. J., Hen R., Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000 101 1 57 66 2-s2.0-0034737299
    • (2000) Cell , vol.101 , Issue.1 , pp. 57-66
    • Yamamoto, A.1    Lucas, J.J.2    Hen, R.3
  • 68
    • 70349469318 scopus 로고    scopus 로고
    • Allele-selective inhibition of mutant huntingtin by peptide nucleic acid-peptide conjugates, locked nucleic acid, and small interfering RNA
    • 2-s2.0-70349469318 10.1111/j.1749-6632.2009.04975.x
    • Hu J., Matsui M., Corey D. R., Allele-selective inhibition of mutant huntingtin by peptide nucleic acid-peptide conjugates, locked nucleic acid, and small interfering RNA. Annals of the New York Academy of Sciences 2009 1175 24 31 2-s2.0-70349469318 10.1111/j.1749-6632.2009.04975.x
    • (2009) Annals of the New York Academy of Sciences , vol.1175 , pp. 24-31
    • Hu, J.1    Matsui, M.2    Corey, D.R.3
  • 69
    • 26844494370 scopus 로고    scopus 로고
    • Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA
    • DOI 10.1016/j.neures.2005.06.021, PII S0168010205001926
    • Wang Y. L., Liu W., Wada E., Murata M., Wada K., Kanazawa I., Clinico-pathological rescue of a model mouse of Huntington's disease by siRNA. Neuroscience Research 2005 53 3 241 249 2-s2.0-26844494370 10.1016/j.neures. 2005.06.021 (Pubitemid 41463777)
    • (2005) Neuroscience Research , vol.53 , Issue.3 , pp. 241-249
    • Wang, Y.-L.1    Liu, W.2    Wada, E.3    Murata, M.4    Wada, K.5    Kanazawa, I.6
  • 71
    • 42549134402 scopus 로고    scopus 로고
    • AAV vector-mediated RNAi of mutant Huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease
    • DOI 10.1038/mt.2008.50, PII MT200850
    • Franich N. R., Fitzsimons H. L., Fong D. M., Klugmann M., During M. J., Young D., AAV vector-mediated RNAi of mutant Huntingtin expression is neuroprotective in a novel genetic rat model of Huntington's disease. Molecular Therapy 2008 16 5 947 956 2-s2.0-42549134402 10.1038/mt.2008.50 (Pubitemid 351587106)
    • (2008) Molecular Therapy , vol.16 , Issue.5 , pp. 947-956
    • Franich, N.R.1    Fitzsimons, H.L.2    Fong, D.M.3    Klugmann, M.4    During, M.J.5    Young, D.6
  • 73
    • 25144464388 scopus 로고    scopus 로고
    • Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice
    • DOI 10.1016/j.ymthe.2005.05.006, PII S1525001605002005
    • Rodriguez-Lebron E., Denovan-Wright E. M., Nash K., Lewin A. S., Mandel R. J., Intrastriatal rAAV-mediated delivery of anti-huntingtin shRNAs induces partial reversal of disease progression in R6/1 Huntington's disease transgenic mice. Molecular Therapy 2005 12 4 618 633 2-s2.0-25144464388 10.1016/j.ymthe.2005.05.006 (Pubitemid 41350143)
    • (2005) Molecular Therapy , vol.12 , Issue.4 , pp. 618-633
    • Rodriguez-Lebron, E.1    Denovan-Wright, E.M.2    Nash, K.3    Lewin, A.S.4    Mandel, R.J.5
  • 75
    • 0029055717 scopus 로고
    • Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
    • 2-s2.0-0029055717
    • Nasir J., Floresco S. B., O'Kusky J. R., Diewert V. M., Richman J. M., Zeisler J., Borowski A., Marth J. D., Phillips A. G., Hayden M. R., Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 1995 81 5 811 823 2-s2.0-0029055717
    • (1995) Cell , vol.81 , Issue.5 , pp. 811-823
    • Nasir, J.1    Floresco, S.B.2    O'Kusky, J.R.3    Diewert, V.M.4    Richman, J.M.5    Zeisler, J.6    Borowski, A.7    Marth, J.D.8    Phillips, A.G.9    Hayden, M.R.10
  • 77
    • 0030613177 scopus 로고    scopus 로고
    • Huntington is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
    • White J. K., Auerbach W., Duyao M. P., Vonsattel J. P., Gusella J. F., Joyner A. L., MacDonald M. E., Huntington is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion. Nature Genetics 1997 17 4 404 410 2-s2.0-0030613177 (Pubitemid 27518388)
    • (1997) Nature Genetics , vol.17 , Issue.4 , pp. 404-410
    • White, J.K.1    Auerbach, W.2    Duyao, M.P.3    Vonsattel, J.-P.4    Gusella, J.F.5    Joyner, A.L.6    MacDonald, M.E.7
  • 78
    • 0023115076 scopus 로고
    • Homozygotes for Huntington's disease
    • DOI 10.1038/326194a0
    • Wexler N. S., Young A. B., Tanzi R. E., Homozygotes for Huntington's disease. Nature 1987 326 6109 194 197 2-s2.0-0023115076 (Pubitemid 17056590)
    • (1987) Nature , vol.326 , Issue.6109 , pp. 194-197
    • Wexler, N.S.1    Young, A.B.2    Tanzi, R.E.3
  • 82
    • 67349100160 scopus 로고    scopus 로고
    • Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice
    • 2-s2.0-67349100160 10.1038/mt.2009.17
    • Boudreau R. L., McBride J. L., Martins I., Shen S., Xing Y., Carter B. J., Davidson B. L., Nonallele-specific silencing of mutant and wild-type huntingtin demonstrates therapeutic efficacy in Huntington's disease mice. Molecular Therapy 2009 17 6 1053 1063 2-s2.0-67349100160 10.1038/mt.2009.17
    • (2009) Molecular Therapy , vol.17 , Issue.6 , pp. 1053-1063
    • Boudreau, R.L.1    McBride, J.L.2    Martins, I.3    Shen, S.4    Xing, Y.5    Carter, B.J.6    Davidson, B.L.7
  • 83
    • 58149232358 scopus 로고    scopus 로고
    • Artificial MicroRNAs as siRNA shuttles: Improved safety as compared to shRNAs in vitro and in vivo
    • 2-s2.0-58149232358 10.1038/mt.2008.231
    • Boudreau R. L., Martins I., Davidson B. L., Artificial MicroRNAs as siRNA shuttles: improved safety as compared to shRNAs in vitro and In vivo. Molecular Therapy 2009 17 1 169 175 2-s2.0-58149232358 10.1038/mt.2008.231
    • (2009) Molecular Therapy , vol.17 , Issue.1 , pp. 169-175
    • Boudreau, R.L.1    Martins, I.2    Davidson, B.L.3
  • 85
    • 78649359013 scopus 로고    scopus 로고
    • Allele-selective inhibition of huntingtin expression by switching to an miRNA-like RNAi mechanism
    • 2-s2.0-78649359013 10.1016/j.chembiol.2010.10.013
    • Hu J., Liu J., Corey D. R., Allele-selective inhibition of huntingtin expression by switching to an miRNA-like RNAi mechanism. Chemistry and Biology 2010 17 11 1183 1188 2-s2.0-78649359013 10.1016/j.chembiol.2010.10.013
    • (2010) Chemistry and Biology , vol.17 , Issue.11 , pp. 1183-1188
    • Hu, J.1    Liu, J.2    Corey, D.R.3
  • 87
    • 57249086448 scopus 로고    scopus 로고
    • Allele-specific silencing of mutant Huntington's disease gene
    • 2-s2.0-57249086448 10.1111/j.1471-4159.2008.05734.x
    • Zhang Y., Engelman J., Friedlander R. M., Allele-specific silencing of mutant Huntington's disease gene. Journal of Neurochemistry 2009 108 1 82 90 2-s2.0-57249086448 10.1111/j.1471-4159.2008.05734.x
    • (2009) Journal of Neurochemistry , vol.108 , Issue.1 , pp. 82-90
    • Zhang, Y.1    Engelman, J.2    Friedlander, R.M.3
  • 88
    • 56149120246 scopus 로고    scopus 로고
    • Linking SNPs to CAG repeat length in Huntington's disease patients
    • 2-s2.0-56149120246 10.1038/nmeth.1261
    • Liu W., Kennington L. A., Rosas H. D., Hersch S., Cha J. H., Zamore P. D., Aronin N., Linking SNPs to CAG repeat length in Huntington's disease patients. Nature Methods 2008 5 11 951 953 2-s2.0-56149120246 10.1038/nmeth.1261
    • (2008) Nature Methods , vol.5 , Issue.11 , pp. 951-953
    • Liu, W.1    Kennington, L.A.2    Rosas, H.D.3    Hersch, S.4    Cha, J.H.5    Zamore, P.D.6    Aronin, N.7
  • 91
    • 34247245632 scopus 로고    scopus 로고
    • Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease
    • DOI 10.1038/mt.sj.6300141, PII 6300141
    • Perrin V., Régulier E., Abbas-Terki T., Hassig R., Brouillet E., Aebischer P., Luthi-Carter R., Déglon N., Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease. Molecular Therapy 2007 15 5 903 911 2-s2.0-34247245632 10.1038/mt.sj.6300141 (Pubitemid 46621648)
    • (2007) Molecular Therapy , vol.15 , Issue.5 , pp. 903-911
    • Perrin, V.1    Regulier, E.2    Abbas-Terki, T.3    Hassig, R.4    Brouillet, E.5    Aebischer, P.6    Luthi-Carter, R.7    Deglon, N.8
  • 92
    • 68949159773 scopus 로고    scopus 로고
    • Developing intrabodies for the therapeutic suppression of neurodegenerative pathology
    • 2-s2.0-68949159773 10.1517/14712590903176387
    • Messer A., Lynch S. M., Butler D. C., Developing intrabodies for the therapeutic suppression of neurodegenerative pathology. Expert Opinion on Biological Therapy 2009 9 9 1189 1197 2-s2.0-68949159773 10.1517/ 14712590903176387
    • (2009) Expert Opinion on Biological Therapy , vol.9 , Issue.9 , pp. 1189-1197
    • Messer, A.1    Lynch, S.M.2    Butler, D.C.3
  • 93
    • 70350543879 scopus 로고    scopus 로고
    • Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease
    • 2-s2.0-70350543879 10.1523/JNEUROSCI.4286-09.2009
    • Southwell A. L., Ko J., Patterson P. H., Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease. Journal of Neuroscience 2009 29 43 13589 13602 2-s2.0-70350543879 10.1523/JNEUROSCI.4286-09.2009
    • (2009) Journal of Neuroscience , vol.29 , Issue.43 , pp. 13589-13602
    • Southwell, A.L.1    Ko, J.2    Patterson, P.H.3
  • 94
    • 34547237479 scopus 로고    scopus 로고
    • Short G-rich oligonucleotides as a potential therapeutic for Huntington's Disease
    • DOI 10.1186/1471-2202-7-65
    • Skogen M., Roth J., Yerkes S., Parekh-Olmedo H., Kmiec E., Short G-rich oligonucleotides as a potential therapeutic for Huntington's Disease. BMC Neuroscience 2006 7, article 65 2-s2.0-34547237479 10.1186/1471-2202-7-65 (Pubitemid 44822885)
    • (2006) BMC Neuroscience , vol.7 , pp. 65
    • Skogen, M.1    Roth, J.2    Yerkes, S.3    Parekh-Olmedo, H.4    Kmiec, E.5
  • 98
    • 1542348209 scopus 로고    scopus 로고
    • The Energetics of Huntington's Disease
    • DOI 10.1023/B:NERE.0000014824.04728.dd
    • Browne S. E., Beal M. F., The energetics of Huntington's disease. Neurochemical Research 2004 29 3 531 546 2-s2.0-1542348209 10.1023/B:NERE. 0000014824.04728.dd (Pubitemid 38295004)
    • (2004) Neurochemical Research , vol.29 , Issue.3 , pp. 531-546
    • Browne, S.E.1    Beal, M.F.2
  • 100
    • 57649187103 scopus 로고    scopus 로고
    • Mitochondria and Huntington's disease pathogenesis: Insight from genetic and chemical models
    • 2-s2.0-57649187103 10.1196/annals.1427.018
    • Browne S. E., Mitochondria and Huntington's disease pathogenesis: insight from genetic and chemical models. Annals of the New York Academy of Sciences 2008 1147 358 382 2-s2.0-57649187103 10.1196/annals.1427.018
    • (2008) Annals of the New York Academy of Sciences , vol.1147 , pp. 358-382
    • Browne, S.E.1
  • 103
    • 0037326196 scopus 로고    scopus 로고
    • Peroxisome proliferator-activated receptor-γ coactivator 1α (PGC-1α): Transcriptional coactivator and metabolic regulator
    • DOI 10.1210/er.2002-0012
    • Puigserver P., Spiegelman B. M., brucespiegelman@dfci.harvard.edu Peroxisome proliferator-activated receptor- γ coactivator 1 α (PGC-1 α): transcriptional coactivator and metabolic regulator. Endocrine Reviews 2003 24 1 78 90 10.1210/er.2002-0012 (Pubitemid 36223280)
    • (2003) Endocrine Reviews , vol.24 , Issue.1 , pp. 78-90
    • Puigserver, P.1    Spiegelman, B.M.2
  • 104
    • 0022343684 scopus 로고
    • Selective sparing of a class of striatal neurons in Huntington's disease
    • Ferrante R. J., Kowall N. W., Beal M. F., Selective sparing of a class of striatal neurons in Huntington's disease. Science 1985 230 4725 561 563 2-s2.0-0022343684 (Pubitemid 16169813)
    • (1985) Science , vol.230 , Issue.4725 , pp. 561-563
    • Ferrante, R.J.1    Kowall, N.W.2    Beal, M.F.3
  • 105
    • 33749042331 scopus 로고    scopus 로고
    • Transcriptional Repression of PGC-1α by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration
    • DOI 10.1016/j.cell.2006.09.015, PII S0092867406012050
    • Cui L., Jeong H., Borovecki F., Parkhurst C. N., Tanese N., Krainc D., Transcriptional repression of PGC-1 α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006 127 1 59 69 2-s2.0-33749042331 10.1016/j.cell.2006.09.015 (Pubitemid 44466642)
    • (2006) Cell , vol.127 , Issue.1 , pp. 59-69
    • Cui, L.1    Jeong, H.2    Borovecki, F.3    Parkhurst, C.N.4    Tanese, N.5    Krainc, D.6
  • 106
    • 33749999530 scopus 로고    scopus 로고
    • Suppression of Reactive Oxygen Species and Neurodegeneration by the PGC-1 Transcriptional Coactivators
    • DOI 10.1016/j.cell.2006.09.024, PII S0092867406012281
    • St-Pierre J., Drori S., Uldry M., Silvaggi J. M., Rhee J., Jäger S., Handschin C., Zheng K., Lin J., Yang W., Simon D. K., Bachoo R., Spiegelman B. M., Suppression of Reactive Oxygen Species and Neurodegeneration by the PGC-1 Transcriptional Coactivators. Cell 2006 127 2 397 408 2-s2.0-33749999530 10.1016/j.cell.2006.09.024 (Pubitemid 44572377)
    • (2006) Cell , vol.127 , Issue.2 , pp. 397-408
    • St-Pierre, J.1    Drori, S.2    Uldry, M.3    Silvaggi, J.M.4    Rhee, J.5    Jager, S.6    Handschin, C.7    Zheng, K.8    Lin, J.9    Yang, W.10    Simon, D.K.11    Bachoo, R.12    Spiegelman, B.M.13
  • 107
    • 32644481227 scopus 로고    scopus 로고
    • Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction
    • DOI 10.1016/j.bbrc.2006.01.136, PII S0006291X06002257
    • Goswami A., Dikshit P., Mishra A., Mulherkar S., Nukina N., Jana N. R., nihar@nbrc.ac.in Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction. Biochemical and Biophysical Research Communications 2006 342 1 184 190 10.1016/j.bbrc.2006.01.136 (Pubitemid 43247375)
    • (2006) Biochemical and Biophysical Research Communications , vol.342 , Issue.1 , pp. 184-190
    • Goswami, A.1    Dikshit, P.2    Mishra, A.3    Mulherkar, S.4    Nukina, N.5    Jana, N.R.6
  • 110
    • 0032450856 scopus 로고    scopus 로고
    • Amyloid formation by mutant huntingtin: Threshold, progressivity and recruitment of normal polyglutamine proteins
    • DOI 10.1023/B:SCAM.0000007124.19463.e5
    • Huang C. C., Faber P. W., Persichetti F., Mittal V., Vonsattel J. P., MacDonald M. E., Gusella J. F., Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins. Somatic Cell and Molecular Genetics 1998 24 4 217 233 2-s2.0-0032450856 10.1023/B:SCAM.0000007124.19463.e5 (Pubitemid 29316230)
    • (1998) Somatic Cell and Molecular Genetics , vol.24 , Issue.4 , pp. 217-233
    • Huang, C.C.1    Faber, P.W.2    Persichetti, F.3    Mittal, V.4    Vonsattel, J.-P.5    MacDonald, M.E.6    Gusella, J.F.7
  • 111
  • 112
  • 113
    • 84855499458 scopus 로고    scopus 로고
    • Huntington's disease: Molecular basis of neurodegeneration
    • 2-s2.0-22344453935
    • Rubinsztein D. C., Carmichael J., Huntington's disease: molecular basis of neurodegeneration. Expert Reviews in Molecular Medicine 2003 5 20 1 21 2-s2.0-22344453935
    • (2003) Expert Reviews in Molecular Medicine , vol.5 , Issue.20 , pp. 1-21
    • Rubinsztein, D.C.1    Carmichael, J.2
  • 114
    • 34347364706 scopus 로고    scopus 로고
    • Widespread disruption of repressor element-1 silencing transcription factor/neuron-restrictive silencer factor occupancy at its target genes in Huntington's disease
    • DOI 10.1523/JNEUROSCI.4278-06.2007
    • Zuccato C., Belyaev N., Conforti P., Ooi L., Tartari M., Papadimou E., MacDonald M., Fossale E., Zeitlin S., Buckley N., Cattaneo E., Widespread disruption of repressor element-1 silencing transcription factor/neuron- restrictive silencer factor occupancy at its target genes in Huntington's disease. Journal of Neuroscience 2007 27 26 6972 6983 2-s2.0-34347364706 10.1523/JNEUROSCI.4278-06.2007 (Pubitemid 47015906)
    • (2007) Journal of Neuroscience , vol.27 , Issue.26 , pp. 6972-6983
    • Zuccato, C.1    Belyaev, N.2    Conforti, P.3    Ooi, L.4    Tartari, M.5    Papadimou, E.6    MacDonald, M.7    Fossale, E.8    Zeitlin, S.9    Buckley, N.10    Cattaneo, E.11
  • 115
    • 78651318398 scopus 로고    scopus 로고
    • Rescue of gene expression by modified REST decoy oligonucleotides in a cellular model of Huntington's disease
    • 10.1111/j.1471-4159.2010.07122.x
    • Soldati C., Bithell A., Conforti P., Cattaneo E., Buckley N. J., noel.buckley@kcl.ac.uk Rescue of gene expression by modified REST decoy oligonucleotides in a cellular model of Huntington's disease. Journal of Neurochemistry 2011 116 3 415 425 10.1111/j.1471-4159.2010.07122.x
    • (2011) Journal of Neurochemistry , vol.116 , Issue.3 , pp. 415-425
    • Soldati, C.1    Bithell, A.2    Conforti, P.3    Cattaneo, E.4    Buckley, N.J.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.