메뉴 건너뛰기




Volumn 556, Issue 2, 2015, Pages 91-97

Huntington's disease: An update of therapeutic strategies

Author keywords

Antisense oligonucleotide (ASO); Histone deacetylase inhibitors (HDACi); Htt gene; Huntington's disease (HD); RNA interference (RNAi); Transglutaminase inhibitors (Tgasei)

Indexed keywords

4 PHENYLBUTYRIC ACID; ACYLTRANSFERASE INHIBITOR; ANTIOXIDANT; ANTISENSE OLIGONUCLEOTIDE; BRAIN DERIVED NEUROTROPHIC FACTOR; CASPASE 6 INHIBITOR; CASPASE INHIBITOR; COMPOUND C2-8; CONGO RED; CREATINE; EPIGALLOCATECHIN GALLATE; GLUTAMIC ACID; HISTONE DEACETYLASE INHIBITOR; HUNTINGTIN; ICOSAPENTAENOIC ACID; IFENPRODIL; LAMOTRIGINE; MEMANTINE; MERCAPTAMINE; MINOCYCLINE; MITHRAMYCIN; N METHYL DEXTRO ASPARTIC ACID RECEPTOR BLOCKING AGENT; RAPAMYCIN; REMACEMIDE; RILUZOLE; SIRTUIN; SYNTHETIC PEPTIDE; TETRABENAZINE; UBIDECARENONE; UNCLASSIFIED DRUG; UNINDEXED DRUG; HTT PROTEIN, HUMAN; NERVE PROTEIN; NEUROPROTECTIVE AGENT;

EID: 84919622781     PISSN: 03781119     EISSN: 18790038     Source Type: Journal    
DOI: 10.1016/j.gene.2014.11.022     Document Type: Review
Times cited : (54)

References (76)
  • 1
    • 0034627143 scopus 로고    scopus 로고
    • Motor and cognitive improvements in patients with Huntington's disease after neural, transplantation
    • Bachoud-Lévi A.C., Rémy P., Nguyen J.P., et al. Motor and cognitive improvements in patients with Huntington's disease after neural, transplantation. Lancet 2000, 356(9246):1975-1979.
    • (2000) Lancet , vol.356 , Issue.9246 , pp. 1975-1979
    • Bachoud-Lévi, A.C.1    Rémy, P.2    Nguyen, J.P.3
  • 2
    • 33644967927 scopus 로고    scopus 로고
    • Effect of fetal neural transplants in patients with Huntington's disease 6years after surgery: a long-term follow-up study
    • Bachoud-Lévi A.C., Gaura V., Brugières P., et al. Effect of fetal neural transplants in patients with Huntington's disease 6years after surgery: a long-term follow-up study. Lancet Neurol. 2006, 5(4):303-309.
    • (2006) Lancet Neurol. , vol.5 , Issue.4 , pp. 303-309
    • Bachoud-Lévi, A.C.1    Gaura, V.2    Brugières, P.3
  • 3
    • 0031918640 scopus 로고    scopus 로고
    • Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length
    • Becher M.W., Kotzuk J.A., Sharp A.H., et al. Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol. Dis. 1998, 4(6):387-397.
    • (1998) Neurobiol. Dis. , vol.4 , Issue.6 , pp. 387-397
    • Becher, M.W.1    Kotzuk, J.A.2    Sharp, A.H.3
  • 4
    • 4444302167 scopus 로고    scopus 로고
    • Deranged neuronal calcium signalling and Huntington disease
    • Bezprozvanny I., Hayden M.R. Deranged neuronal calcium signalling and Huntington disease. Biochem. Biophys. Res. Commun. 2004, 322:1310-1317.
    • (2004) Biochem. Biophys. Res. Commun. , vol.322 , pp. 1310-1317
    • Bezprozvanny, I.1    Hayden, M.R.2
  • 5
    • 20444444649 scopus 로고    scopus 로고
    • Mechanism of human SIRT1 activation by resveratrol
    • Borra M.T., Smith B.C., Denu J.M. Mechanism of human SIRT1 activation by resveratrol. J. Biol. Chem. 2005, 280:17187-17195.
    • (2005) J. Biol. Chem. , vol.280 , pp. 17187-17195
    • Borra, M.T.1    Smith, B.C.2    Denu, J.M.3
  • 6
    • 33746687898 scopus 로고    scopus 로고
    • Clinical experience with risperidone and memantine in the treatment of Huntington's disease
    • Cankurtaran E.S., Ozalp E., Soygur H., et al. Clinical experience with risperidone and memantine in the treatment of Huntington's disease. J. Natl. Med. Assoc. 2006, 98:1353-1355.
    • (2006) J. Natl. Med. Assoc. , vol.98 , pp. 1353-1355
    • Cankurtaran, E.S.1    Ozalp, E.2    Soygur, H.3
  • 8
    • 0034425217 scopus 로고    scopus 로고
    • Structural basis of DNA recognition by anticancer antibiotics, chromomycin A(3), mithramycin: roles of minor groove width and ligand flexibility
    • Chakrabarti S., Bhattacharyya D., Dasgupta D. Structural basis of DNA recognition by anticancer antibiotics, chromomycin A(3), mithramycin: roles of minor groove width and ligand flexibility. Biopolymers 2000, 56:85-95.
    • (2000) Biopolymers , vol.56 , pp. 85-95
    • Chakrabarti, S.1    Bhattacharyya, D.2    Dasgupta, D.3
  • 9
    • 0033912716 scopus 로고    scopus 로고
    • Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease
    • Chen M., Ona V.O., Li M., et al. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat. Med. 2000, 6:797-801.
    • (2000) Nat. Med. , vol.6 , pp. 797-801
    • Chen, M.1    Ona, V.O.2    Li, M.3
  • 10
    • 3543141113 scopus 로고    scopus 로고
    • Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release
    • Choo Y.S., Johnson G.V., MacDonald M., Detloff P.J., Lesort M. Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum. Mol. Genet. 2004, 13:1407-1420.
    • (2004) Hum. Mol. Genet. , vol.13 , pp. 1407-1420
    • Choo, Y.S.1    Johnson, G.V.2    MacDonald, M.3    Detloff, P.J.4    Lesort, M.5
  • 11
    • 36749022180 scopus 로고    scopus 로고
    • A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse
    • Chopra V., Fox J.H., Lieberman G., et al. A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse. Proc. Natl. Acad. Sci. U. S. A. 2007, 104:16685-16689.
    • (2007) Proc. Natl. Acad. Sci. U. S. A. , vol.104 , pp. 16685-16689
    • Chopra, V.1    Fox, J.H.2    Lieberman, G.3
  • 12
    • 42749089265 scopus 로고    scopus 로고
    • Neurological disease
    • W.B. Saunders, Edinburgh and New York, P. Kumar, M. Clark (Eds.)
    • Clarke C.R.A. Neurological disease. Kumar and Clark Clinical Medicine 2005, 1173-1271. W.B. Saunders, Edinburgh and New York. 6th ed. P. Kumar, M. Clark (Eds.).
    • (2005) Kumar and Clark Clinical Medicine , pp. 1173-1271
    • Clarke, C.R.A.1
  • 13
    • 0030752709 scopus 로고    scopus 로고
    • Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuritis in brain
    • DiFiglia M., Sapp E., Chase K.O., et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neuritis in brain. Science 1997, 277:1990-1993.
    • (1997) Science , vol.277 , pp. 1990-1993
    • DiFiglia, M.1    Sapp, E.2    Chase, K.O.3
  • 14
    • 33748744375 scopus 로고    scopus 로고
    • Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models
    • Ehrnhoefer D.E., Duennwald M., Markovic P., et al. Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models. Hum. Mol. Genet. 2006, 15:2743-2751.
    • (2006) Hum. Mol. Genet. , vol.15 , pp. 2743-2751
    • Ehrnhoefer, D.E.1    Duennwald, M.2    Markovic, P.3
  • 15
    • 0034660457 scopus 로고    scopus 로고
    • Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease
    • Ferrante R.J., Andreassen O.A., Jenkins B.G., et al. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J. Neurosci. 2000, 20:4389-4397.
    • (2000) J. Neurosci. , vol.20 , pp. 4389-4397
    • Ferrante, R.J.1    Andreassen, O.A.2    Jenkins, B.G.3
  • 16
    • 0036523110 scopus 로고    scopus 로고
    • Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease
    • Ferrante R.J., Andreassen O.A., Dedeoglu A., et al. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J. Neurosci. 2002, 22:1592-1599.
    • (2002) J. Neurosci. , vol.22 , pp. 1592-1599
    • Ferrante, R.J.1    Andreassen, O.A.2    Dedeoglu, A.3
  • 17
    • 0027510177 scopus 로고
    • Pre- and postsynaptic neurotoxic effects of dopamine demonstrated by intrastriatal injection
    • Filloux F., Townsend J.J. Pre- and postsynaptic neurotoxic effects of dopamine demonstrated by intrastriatal injection. Exp. Neurol. 1993, 119:79-88.
    • (1993) Exp. Neurol. , vol.119 , pp. 79-88
    • Filloux, F.1    Townsend, J.J.2
  • 19
    • 84856326544 scopus 로고    scopus 로고
    • Protein synthesis: translation and posttranslational modifications
    • John Wiley & Sons, Inc., Hoboken NJ, T.M. Devlin (Ed.)
    • Glitz D. Protein synthesis: translation and posttranslational modifications. Textbook of Biochemistry: With Clinical Correlations 2011, 209-254. John Wiley & Sons, Inc., Hoboken NJ. 7th ed. T.M. Devlin (Ed.).
    • (2011) Textbook of Biochemistry: With Clinical Correlations , pp. 209-254
    • Glitz, D.1
  • 20
    • 33745003424 scopus 로고    scopus 로고
    • Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin
    • Graham R.K., Deng Y., Slow E.J., et al. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 2006, 125:1179-1191.
    • (2006) Cell , vol.125 , pp. 1179-1191
    • Graham, R.K.1    Deng, Y.2    Slow, E.J.3
  • 21
    • 0036901574 scopus 로고    scopus 로고
    • Transglutaminases: nature's biological glues
    • Griffin M., Casadio R., Bergamini C.M. Transglutaminases: nature's biological glues. Biochem. J. 2002, 368(2):377-396.
    • (2002) Biochem. J. , vol.368 , Issue.2 , pp. 377-396
    • Griffin, M.1    Casadio, R.2    Bergamini, C.M.3
  • 22
    • 0030764219 scopus 로고    scopus 로고
    • Riluzole protects from motor deficits and striatal degeneration produced by systemic 3-nitropropionic acid intoxication in rats
    • Guyot M.C., Palfi S., Stutzmann J.M., et al. Riluzole protects from motor deficits and striatal degeneration produced by systemic 3-nitropropionic acid intoxication in rats. Neuroscience 1997, 81:141-149.
    • (1997) Neuroscience , vol.81 , pp. 141-149
    • Guyot, M.C.1    Palfi, S.2    Stutzmann, J.M.3
  • 23
    • 30344476118 scopus 로고    scopus 로고
    • Pharmaceutical, cellular and genetic therapies for Huntington's disease
    • Handley O.J., Naji J.J., Dunnett S.B., et al. Pharmaceutical, cellular and genetic therapies for Huntington's disease. Clin. Sci. 2006, 110:73-88.
    • (2006) Clin. Sci. , vol.110 , pp. 73-88
    • Handley, O.J.1    Naji, J.J.2    Dunnett, S.B.3
  • 24
    • 30344476118 scopus 로고    scopus 로고
    • Pharmaceutical, cellular and genetic therapies for Huntington's disease
    • Handley O.J., Naji J.J., Dunnett S.B., Rosser A.E. Pharmaceutical, cellular and genetic therapies for Huntington's disease. Clin. Sci. 2006, 110:73-88.
    • (2006) Clin. Sci. , vol.110 , pp. 73-88
    • Handley, O.J.1    Naji, J.J.2    Dunnett, S.B.3    Rosser, A.E.4
  • 25
    • 20244378556 scopus 로고    scopus 로고
    • RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model
    • Harper S.Q., Staber P.D., He X., et al. RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. Proc. Natl. Acad. Sci. U. S. A. 2005, 102:5820-5825.
    • (2005) Proc. Natl. Acad. Sci. U. S. A. , vol.102 , pp. 5820-5825
    • Harper, S.Q.1    Staber, P.D.2    He, X.3
  • 26
    • 10744228913 scopus 로고    scopus 로고
    • Minocycline is protective in a mouse model of Huntington's disease
    • Hersch S., Fink K., Vonsattel J.P., et al. Minocycline is protective in a mouse model of Huntington's disease. Ann. Neurol. 2003, 54:841-843.
    • (2003) Ann. Neurol. , vol.54 , pp. 841-843
    • Hersch, S.1    Fink, K.2    Vonsattel, J.P.3
  • 27
    • 33644927838 scopus 로고    scopus 로고
    • Creatine in Huntington disease is safe, tolerable, and bioavailable in brain and reduces serum 8OH2'dG
    • Hersch S.M., Gevorkian S., Marder K., et al. Creatine in Huntington disease is safe, tolerable, and bioavailable in brain and reduces serum 8OH2'dG. Neurology 2006, 66:250-252.
    • (2006) Neurology , vol.66 , pp. 250-252
    • Hersch, S.M.1    Gevorkian, S.2    Marder, K.3
  • 28
    • 0037452775 scopus 로고    scopus 로고
    • Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease
    • Hockly E., Richon V.M., Woodman B., et al. Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease. Proc. Natl. Acad. Sci. U. S. A. 2003, 100:2041-2046.
    • (2003) Proc. Natl. Acad. Sci. U. S. A. , vol.100 , pp. 2041-2046
    • Hockly, E.1    Richon, V.M.2    Woodman, B.3
  • 29
    • 0029431871 scopus 로고
    • Marked increase in mitochondrial DNA deletion levels in the cerebral cortex of Huntington's disease patients
    • Horton T.M., Graham B.H., Corral-Debrinski M., Shoffner J.M., Kaufman A.E., Beal M.F. Marked increase in mitochondrial DNA deletion levels in the cerebral cortex of Huntington's disease patients. Neurology 1995, 45:1879-1883.
    • (1995) Neurology , vol.45 , pp. 1879-1883
    • Horton, T.M.1    Graham, B.H.2    Corral-Debrinski, M.3    Shoffner, J.M.4    Kaufman, A.E.5    Beal, M.F.6
  • 30
    • 70349469318 scopus 로고    scopus 로고
    • Allele-selective inhibition of mutant huntingtin by peptide nucleic acid-peptide conjugates, locked nucleic acid, and small interfering RNA
    • 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. Ann. N. Y. Acad. Sci. 2009, 1175:24-31.
    • (2009) Ann. N. Y. Acad. Sci. , vol.1175 , pp. 24-31
    • Hu, J.1    Matsui, M.2    Corey, D.R.3
  • 31
    • 28844475400 scopus 로고    scopus 로고
    • HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin
    • Iwata A., Riley B.E., Johnston J.A., et al. HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin. J. Biol. Chem. 2005, 280:40282-40292.
    • (2005) J. Biol. Chem. , vol.280 , pp. 40282-40292
    • Iwata, A.1    Riley, B.E.2    Johnston, J.A.3
  • 32
    • 0034212341 scopus 로고    scopus 로고
    • Neuronal cell death in Huntington's disease: a potential role for dopamine
    • Jakel R.J., Maragos W.F. Neuronal cell death in Huntington's disease: a potential role for dopamine. Trends Neurosci. 2000, 23:239-245.
    • (2000) Trends Neurosci. , vol.23 , pp. 239-245
    • Jakel, R.J.1    Maragos, W.F.2
  • 33
    • 0036172346 scopus 로고    scopus 로고
    • Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine
    • Karpuj M.V., Becher M.W., Springer J.E., et al. Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat. Med. 2002, 8:143-149.
    • (2002) Nat. Med. , vol.8 , pp. 143-149
    • Karpuj, M.V.1    Becher, M.W.2    Springer, J.E.3
  • 34
    • 0036134819 scopus 로고    scopus 로고
    • Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications
    • Karpuj M.V., Becher M.W., Steinman L. Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications. Neurochem. Int. 2002, 40(1):31-36.
    • (2002) Neurochem. Int. , vol.40 , Issue.1 , pp. 31-36
    • Karpuj, M.V.1    Becher, M.W.2    Steinman, L.3
  • 35
    • 9244263519 scopus 로고    scopus 로고
    • A controlled trial of remacemide hydrochloride in Huntington's disease
    • Kieburtz K., Feigin A., McDermott M., et al. A controlled trial of remacemide hydrochloride in Huntington's disease. Mov. Disord. 1996, 11:273-277.
    • (1996) Mov. Disord. , vol.11 , pp. 273-277
    • Kieburtz, K.1    Feigin, A.2    McDermott, M.3
  • 36
    • 0037019332 scopus 로고    scopus 로고
    • Dopamine neurons derived from embryonic stem cells function in an animal model of Parkinson's disease
    • Kim J.H., Auerbach J.M., Rodriguez-Gomez J.A., et al. Dopamine neurons derived from embryonic stem cells function in an animal model of Parkinson's disease. Nature 2002, 418:50-56.
    • (2002) Nature , vol.418 , pp. 50-56
    • Kim, J.H.1    Auerbach, J.M.2    Rodriguez-Gomez, J.A.3
  • 37
    • 0028316870 scopus 로고
    • A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats
    • Kremer B., Goldberg P., Andrew S.E., et al. A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats. N. Engl. J. Med. 1994, 330:1401-1406.
    • (1994) N. Engl. J. Med. , vol.330 , pp. 1401-1406
    • Kremer, B.1    Goldberg, P.2    Andrew, S.E.3
  • 38
    • 0033595502 scopus 로고    scopus 로고
    • Influence of lamotrigine on progression of early Huntington disease: a randomized clinical trial
    • Kremer B., Clark C.M., Almqvist E.W., et al. Influence of lamotrigine on progression of early Huntington disease: a randomized clinical trial. Neurology 1999, 53:1000-1011.
    • (1999) Neurology , vol.53 , pp. 1000-1011
    • Kremer, B.1    Clark, C.M.2    Almqvist, E.W.3
  • 39
    • 33750378660 scopus 로고    scopus 로고
    • Memantine reduces striatal cell death with decreasing calpain level in 3-nitropropionic model of Huntington's disease
    • Lee S.T., Chu K., Park J.E., et al. Memantine reduces striatal cell death with decreasing calpain level in 3-nitropropionic model of Huntington's disease. Brain Res. 2006, 1118:199-207.
    • (2006) Brain Res. , vol.1118 , pp. 199-207
    • Lee, S.T.1    Chu, K.2    Park, J.E.3
  • 40
    • 33847241277 scopus 로고    scopus 로고
    • Trinucleotide repeat disorders
    • Lutz R.E. Trinucleotide repeat disorders. Semin. Pediatr. Neurol. 2007, 14(1):26-33.
    • (2007) Semin. Pediatr. Neurol. , vol.14 , Issue.1 , pp. 26-33
    • Lutz, R.E.1
  • 41
    • 33645450264 scopus 로고    scopus 로고
    • Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells
    • Mao Z., Choo Y.S., Lesort M. Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells. Eur. J. Neurosci. 2006, 23:1701-1710.
    • (2006) Eur. J. Neurosci. , vol.23 , pp. 1701-1710
    • Mao, Z.1    Choo, Y.S.2    Lesort, M.3
  • 42
    • 0028803390 scopus 로고
    • Effect of riluzole on quinolinate induced neuronal damage in rats: comparison with blockers of glutamatergic neurotransmission
    • Mary V., Wahl F., Stutzmann J.M. Effect of riluzole on quinolinate induced neuronal damage in rats: comparison with blockers of glutamatergic neurotransmission. Neurosci. Lett. 1995, 201:92-96.
    • (1995) Neurosci. Lett. , vol.201 , pp. 92-96
    • Mary, V.1    Wahl, F.2    Stutzmann, J.M.3
  • 44
    • 0029055717 scopus 로고
    • Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioural and morphological changes in heterozygotes
    • Nasir J., Floresco S.B., O'Kusky J.R., et al. Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioural and morphological changes in heterozygotes. Cell 1995, 81(5):811-823.
    • (1995) Cell , vol.81 , Issue.5 , pp. 811-823
    • Nasir, J.1    Floresco, S.B.2    O'Kusky, J.R.3
  • 45
    • 0033626428 scopus 로고    scopus 로고
    • Inhibition of Huntington synthesis by antisense oligodeoxynucleotides
    • Nellemann C., Abell K., Norremolle A., et al. Inhibition of Huntington synthesis by antisense oligodeoxynucleotides. Mol. Cell. Neurosci. 2000, 16:313-323.
    • (2000) Mol. Cell. Neurosci. , vol.16 , pp. 313-323
    • Nellemann, C.1    Abell, K.2    Norremolle, A.3
  • 46
    • 0037412811 scopus 로고    scopus 로고
    • Neurodegenerative disorders: George Huntington's description of hereditary chorea
    • Neylan T.C. Neurodegenerative disorders: George Huntington's description of hereditary chorea. J. Neuropsychiatry Clin. Neurosci. 2003, 15(1):108.
    • (2003) J. Neuropsychiatry Clin. Neurosci. , vol.15 , Issue.1 , pp. 108
    • Neylan, T.C.1
  • 47
    • 40849147435 scopus 로고    scopus 로고
    • NH2-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking
    • Orr A.L., Li S., Wang C.E., Li H., Wang J., Rong J. NH2-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking. J. Neurosci. 2008, 28:2783-2792.
    • (2008) J. Neurosci. , vol.28 , pp. 2783-2792
    • Orr, A.L.1    Li, S.2    Wang, C.E.3    Li, H.4    Wang, J.5    Rong, J.6
  • 48
    • 0031193501 scopus 로고    scopus 로고
    • Hantraye P. Riluzole reduces incidence of abnormal movements but not striatal cell death in a primate model of progressive striatal degeneration
    • Palfi S., Riche D., Brouillet E., et al. Hantraye P. Riluzole reduces incidence of abnormal movements but not striatal cell death in a primate model of progressive striatal degeneration. Exp. Neurol. 1997, 146:135-141.
    • (1997) Exp. Neurol. , vol.146 , pp. 135-141
    • Palfi, S.1    Riche, D.2    Brouillet, E.3
  • 49
    • 0015790444 scopus 로고
    • Huntington's chorea. Deficiency of gamma-aminobutyric acid in brain
    • Perry T.L., Hansen S., Kloster M. Huntington's chorea. Deficiency of gamma-aminobutyric acid in brain. N. Engl. J. Med. 1973, 288:337-342.
    • (1973) N. Engl. J. Med. , vol.288 , pp. 337-342
    • Perry, T.L.1    Hansen, S.2    Kloster, M.3
  • 50
    • 27744478499 scopus 로고    scopus 로고
    • Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease
    • Raamsdonk V.J.M., Pearson J., Rogers D.A., et al. Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease. Exp. Neurol. 2005, 196:266-272.
    • (2005) Exp. Neurol. , vol.196 , pp. 266-272
    • Raamsdonk, V.J.M.1    Pearson, J.2    Rogers, D.A.3
  • 51
    • 2642586352 scopus 로고    scopus 로고
    • Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease
    • Ravikumar B., Vacher C., Berger Z., et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat. Genet. 2004, 36:585-595.
    • (2004) Nat. Genet. , vol.36 , pp. 585-595
    • Ravikumar, B.1    Vacher, C.2    Berger, Z.3
  • 52
    • 0034101804 scopus 로고    scopus 로고
    • Embryonic stem cell lines from human blastocysts: somatic differentiation in vitro
    • Reubinoff B.E., Pera M.F., Fong C.Y., et al. Embryonic stem cell lines from human blastocysts: somatic differentiation in vitro. Nat. Biotechnol. 2000, 18:399-404.
    • (2000) Nat. Biotechnol. , vol.18 , pp. 399-404
    • Reubinoff, B.E.1    Pera, M.F.2    Fong, C.Y.3
  • 54
    • 0037461730 scopus 로고    scopus 로고
    • Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders
    • Sanchez I., Mahlke C., Yuan J. Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 2003, 421:373-379.
    • (2003) Nature , vol.421 , pp. 373-379
    • Sanchez, I.1    Mahlke, C.2    Yuan, J.3
  • 55
    • 34247161367 scopus 로고    scopus 로고
    • A novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein
    • Sarkar S., Davies J.E., Huang Z., et al. A novel mTOR-independent autophagy enhancer, accelerates the clearance of mutant huntingtin and alpha-synuclein. J. Biol. Chem. 2007, 282:5641-5652.
    • (2007) J. Biol. Chem. , vol.282 , pp. 5641-5652
    • Sarkar, S.1    Davies, J.E.2    Huang, Z.3
  • 56
    • 37849042536 scopus 로고    scopus 로고
    • A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin
    • Sarkar S., Krishna G., Imarisio S., et al. A rational mechanism for combination treatment of Huntington's disease using lithium and rapamycin. Hum. Mol. Genet. 2008, 17:170-178.
    • (2008) Hum. Mol. Genet. , vol.17 , pp. 170-178
    • Sarkar, S.1    Krishna, G.2    Imarisio, S.3
  • 57
    • 0035960544 scopus 로고    scopus 로고
    • Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model
    • Schilling G., Coonfield M.L., Ross C.A., et al. Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model. Neurosci. Lett. 2001, 315:149-153.
    • (2001) Neurosci. Lett. , vol.315 , pp. 149-153
    • Schilling, G.1    Coonfield, M.L.2    Ross, C.A.3
  • 58
    • 0032506231 scopus 로고    scopus 로고
    • Derivation of pluripotent stem cells from cultured human primordial germ cells
    • Shamblott M.J., Axelman J., Wang S., et al. Derivation of pluripotent stem cells from cultured human primordial germ cells. Proc. Natl. Acad. Sci. U. S. A. 1998, 95:13726-13731.
    • (1998) Proc. Natl. Acad. Sci. U. S. A. , vol.95 , pp. 13726-13731
    • Shamblott, M.J.1    Axelman, J.2    Wang, S.3
  • 59
    • 70350543879 scopus 로고    scopus 로고
    • Intrabody gene therapy ameliorates motor, cognitive, and neuropathological symptoms in multiple mouse models of Huntington's disease
    • 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. J. Neurosci. 2009, 29:13589-13602.
    • (2009) J. Neurosci. , vol.29 , pp. 13589-13602
    • Southwell, A.L.1    Ko, J.2    Patterson, P.H.3
  • 60
    • 0018973928 scopus 로고
    • Neurochemical alterations in Huntington's chorea: a study of post-mortem brain tissue
    • Spokes E.G. Neurochemical alterations in Huntington's chorea: a study of post-mortem brain tissue. Brain 1980, 103:179-210.
    • (1980) Brain , vol.103 , pp. 179-210
    • Spokes, E.G.1
  • 62
    • 1642633757 scopus 로고    scopus 로고
    • Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease
    • Tanaka M., Machida Y., Niu S., et al. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat. Med. 2004, 10:148-154.
    • (2004) Nat. Med. , vol.10 , pp. 148-154
    • Tanaka, M.1    Machida, Y.2    Niu, S.3
  • 63
    • 34547411294 scopus 로고    scopus 로고
    • Dopaminergic signalling and striatal neurodegeneration in Huntington's disease
    • Tang T.S., Chen X., Liu J., et al. Dopaminergic signalling and striatal neurodegeneration in Huntington's disease. J. Neurosci. 2007, 27:7899-7910.
    • (2007) J. Neurosci. , vol.27 , pp. 7899-7910
    • Tang, T.S.1    Chen, X.2    Liu, J.3
  • 64
    • 33645798913 scopus 로고    scopus 로고
    • Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial
    • The HDCRG Tetrabenazine as antichorea therapy in Huntington disease: a randomized controlled trial. Neurology 2006, 66:366-372.
    • (2006) Neurology , vol.66 , pp. 366-372
  • 65
    • 55749103407 scopus 로고    scopus 로고
    • The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice
    • Thomas E.A., Coppola G., Desplats P.A., et al. The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice. Proc. Natl. Acad. Sci. U. S. A. 2008, 105:15564-15569.
    • (2008) Proc. Natl. Acad. Sci. U. S. A. , vol.105 , pp. 15564-15569
    • Thomas, E.A.1    Coppola, G.2    Desplats, P.A.3
  • 67
    • 33846351654 scopus 로고    scopus 로고
    • Selective inhibitors of death in mutant huntingtin cells
    • Varma H., Voisine C., Demarco C.T., et al. Selective inhibitors of death in mutant huntingtin cells. Nat. Chem. Biol. 2007, 3:99-100.
    • (2007) Nat. Chem. Biol. , vol.3 , pp. 99-100
    • Varma, H.1    Voisine, C.2    Demarco, C.T.3
  • 68
    • 7244254372 scopus 로고    scopus 로고
    • Elevated concentration of cerebrospinal fluid tissue transglutaminase in Parkinson's disease indicating apoptosis
    • Verme I., Steur E.N., Jirikowski G.F., et al. Elevated concentration of cerebrospinal fluid tissue transglutaminase in Parkinson's disease indicating apoptosis. Mov. Disord. 2004, 19(10):1252-1254.
    • (2004) Mov. Disord. , vol.19 , Issue.10 , pp. 1252-1254
    • Verme, I.1    Steur, E.N.2    Jirikowski, G.F.3
  • 69
    • 78650572673 scopus 로고    scopus 로고
    • Mootha Meclizine is neuroprotective in models of Huntington's disease
    • Vishal M.G., Nicolas Offner, James A., et al. Mootha Meclizine is neuroprotective in models of Huntington's disease. Hum. Mol. Genet. 2011, 20(2):294-300.
    • (2011) Hum. Mol. Genet. , vol.20 , Issue.2 , pp. 294-300
    • Vishal, M.G.1    Nicolas, O.2    James, A.3
  • 70
    • 33644771265 scopus 로고    scopus 로고
    • Suppression of polyglutamine-induced toxicity in cell and animal models of Huntington's disease by ubiquilin
    • Wang H., Lim P.J., Yin C., et al. Suppression of polyglutamine-induced toxicity in cell and animal models of Huntington's disease by ubiquilin. Hum. Mol. Genet. 2006, 15(6):1025-1041.
    • (2006) Hum. Mol. Genet. , vol.15 , Issue.6 , pp. 1025-1041
    • Wang, H.1    Lim, P.J.2    Yin, C.3
  • 71
    • 0037047320 scopus 로고    scopus 로고
    • Directed differentiation of embryonic stem cells into motor neurons
    • Wichterle H., Lieberam I., Porter J.A., et al. Directed differentiation of embryonic stem cells into motor neurons. Cell 2002, 110:385-397.
    • (2002) Cell , vol.110 , pp. 385-397
    • Wichterle, H.1    Lieberam, I.2    Porter, J.A.3
  • 72
    • 65549091910 scopus 로고    scopus 로고
    • Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases
    • Lichuan Yang, Calingasan Noel Y., Wille Elizabeth J., et al. Combination therapy with coenzyme Q10 and creatine produces additive neuroprotective effects in models of Parkinson's and Huntington's diseases. J. Neurochem. 2009, 109(5):1427-1439.
    • (2009) J. Neurochem. , vol.109 , Issue.5 , pp. 1427-1439
    • Lichuan, Y.1    Calingasan, N.Y.2    Wille, E.J.3
  • 73
    • 33745658384 scopus 로고    scopus 로고
    • Oxford University Press, Oxford
    • Young I.D. Medical Genetics 2005, Oxford University Press, Oxford.
    • (2005) Medical Genetics
    • Young, I.D.1
  • 74
    • 0037075624 scopus 로고    scopus 로고
    • Increased sensitivity to N-methyl-d-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease
    • Zeron M.M., Hansson O., Chen N., Wellington C.L., et al. Increased sensitivity to N-methyl-d-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron 2002, 33:849-860.
    • (2002) Neuron , vol.33 , pp. 849-860
    • Zeron, M.M.1    Hansson, O.2    Chen, N.3    Wellington, C.L.4
  • 75
    • 0035200623 scopus 로고    scopus 로고
    • In vitro differentiation of transplantable neural precursors from human embryonic stem cells
    • Zhang S.C., Wernig M., Duncan I.D., et al. In vitro differentiation of transplantable neural precursors from human embryonic stem cells. Nat. Biotechnol. 2001, 19:1129-1133.
    • (2001) Nat. Biotechnol. , vol.19 , pp. 1129-1133
    • Zhang, S.C.1    Wernig, M.2    Duncan, I.D.3
  • 76
    • 0035919701 scopus 로고    scopus 로고
    • Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
    • Zuccato C., Ciammola A., Rigamonti D., et al. Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 2001, 293(5529):493-498.
    • (2001) Science , vol.293 , Issue.5529 , pp. 493-498
    • Zuccato, C.1    Ciammola, A.2    Rigamonti, D.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.