메뉴 건너뛰기




Volumn 229, Issue 2, 2012, Pages 308-319

Neuronal aggregates are associated with phenotypic onset in the R6/2 Huntington's disease transgenic mouse

Author keywords

Aggregation; CAG repeats; Huntington's disease; Polyglutamine; R6 2 mouse

Indexed keywords

HUNTINGTIN; MUTANT PROTEIN; POLYGLUTAMINE; TRINUCLEOTIDE;

EID: 84857784547     PISSN: 01664328     EISSN: 18727549     Source Type: Journal    
DOI: 10.1016/j.bbr.2011.12.045     Document Type: Article
Times cited : (12)

References (83)
  • 1
    • 0027176364 scopus 로고
    • The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease
    • Andrew S.E., Goldberg Y.P., Kremer B., Telenius H., Theilmann J., Adam S., et al. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease. Nat Genet 1993, 4:398-403.
    • (1993) Nat Genet , vol.4 , pp. 398-403
    • Andrew, S.E.1    Goldberg, Y.P.2    Kremer, B.3    Telenius, H.4    Theilmann, J.5    Adam, S.6
  • 2
    • 0027240431 scopus 로고
    • Trinucleotide repeat length instability and age of onset in Huntington's disease
    • Duyao M., Ambrose C., Myers R., Novelletto A., Persichetti F., Frontali M., et al. Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet 1993, 4:387-392.
    • (1993) Nat Genet , vol.4 , pp. 387-392
    • Duyao, M.1    Ambrose, C.2    Myers, R.3    Novelletto, A.4    Persichetti, F.5    Frontali, M.6
  • 3
    • 0027261537 scopus 로고
    • Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease
    • Snell R.G., MacMillan J.C., Cheadle J.P., Fenton I., Lazarou L.P., Davies P., et al. Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease. Nat Genet 1993, 4:393-397.
    • (1993) Nat Genet , vol.4 , pp. 393-397
    • Snell, R.G.1    MacMillan, J.C.2    Cheadle, J.P.3    Fenton, I.4    Lazarou, L.P.5    Davies, P.6
  • 4
    • 0027377151 scopus 로고
    • Correlation between the onset age of Huntington's disease and length of the trinucleotide repeat in IT-15
    • Stine O.C., Pleasant N., Franz M.L., Abbott M.H., Folstein S.E., Ross C.A. Correlation between the onset age of Huntington's disease and length of the trinucleotide repeat in IT-15. Hum Mol Genet 1993, 2:1547-1549.
    • (1993) Hum Mol Genet , vol.2 , pp. 1547-1549
    • Stine, O.C.1    Pleasant, N.2    Franz, M.L.3    Abbott, M.H.4    Folstein, S.E.5    Ross, C.A.6
  • 5
    • 0030935035 scopus 로고    scopus 로고
    • The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size
    • Brinkman R.R., Mezei M.M., Theilmann J., Almqvist E., Hayden M.R. The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size. Am J Hum Genet 1997, 60:1202-1210.
    • (1997) Am J Hum Genet , vol.60 , pp. 1202-1210
    • Brinkman, R.R.1    Mezei, M.M.2    Theilmann, J.3    Almqvist, E.4    Hayden, M.R.5
  • 6
    • 0027381482 scopus 로고
    • Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent
    • Telenius H., Kremer H.P., Theilmann J., Andrew S.E., Almqvist E., Anvret M., et al. Molecular analysis of juvenile Huntington disease: the major influence on (CAG)n repeat length is the sex of the affected parent. Hum Mol Genet 1993, 2:1535-1540.
    • (1993) Hum Mol Genet , vol.2 , pp. 1535-1540
    • Telenius, H.1    Kremer, H.P.2    Theilmann, J.3    Andrew, S.E.4    Almqvist, E.5    Anvret, M.6
  • 7
    • 0036756963 scopus 로고    scopus 로고
    • CAG mutation effect on rate of progression in Huntington's disease
    • Squitieri F., Cannella M., Simonelli M. CAG mutation effect on rate of progression in Huntington's disease. Neurol Sci 2002, 23(Suppl. 2):S107-S108.
    • (2002) Neurol Sci , vol.23 , Issue.SUPPL. 2
    • Squitieri, F.1    Cannella, M.2    Simonelli, M.3
  • 8
    • 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 1998, 53:233-242.
    • (1998) Clin Genet , vol.53 , pp. 233-242
    • Hackam, A.S.1    Wellington, C.L.2    Hayden, M.R.3
  • 9
    • 17344363559 scopus 로고    scopus 로고
    • Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
    • Martindale D., Hackam A., Wieczorek A., Ellerby L., Wellington C., McCutcheon K., et al. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet 1998, 18:150-154.
    • (1998) Nat Genet , vol.18 , pp. 150-154
    • Martindale, D.1    Hackam, A.2    Wieczorek, A.3    Ellerby, L.4    Wellington, C.5    McCutcheon, K.6
  • 10
    • 0037015081 scopus 로고    scopus 로고
    • Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation
    • Chen S., Ferrone F.A., Wetzel R. Huntington's disease age-of-onset linked to polyglutamine aggregation nucleation. Proc Natl Acad Sci USA 2002, 99:11884-11889.
    • (2002) Proc Natl Acad Sci USA , vol.99 , pp. 11884-11889
    • Chen, S.1    Ferrone, F.A.2    Wetzel, R.3
  • 11
    • 34548510580 scopus 로고    scopus 로고
    • The length dependence of the polyQ-mediated protein aggregation
    • Barton S., Jacak R., Khare S.D., Ding F., Dokholyan N.V. The length dependence of the polyQ-mediated protein aggregation. J Biol Chem 2007, 282:25487-25492.
    • (2007) J Biol Chem , vol.282 , pp. 25487-25492
    • Barton, S.1    Jacak, R.2    Khare, S.D.3    Ding, F.4    Dokholyan, N.V.5
  • 12
    • 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
    • Group HsDCR
    • Group HsDCR 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, 72:971-983.
    • (1993) Cell , vol.72 , pp. 971-983
  • 13
    • 20744433946 scopus 로고    scopus 로고
    • Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's disease
    • Brustovetsky N., LaFrance R., Purl K.J., Brustovetsky T., Keene C.D., Low W.C., et al. Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's disease. J Neurochem 2005, 93:1361-1370.
    • (2005) J Neurochem , vol.93 , pp. 1361-1370
    • Brustovetsky, N.1    LaFrance, R.2    Purl, K.J.3    Brustovetsky, T.4    Keene, C.D.5    Low, W.C.6
  • 14
    • 0033136692 scopus 로고    scopus 로고
    • A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
    • Hodgson J.G., Agopyan N., Gutekunst C.A., Leavitt B.R., LePiane F., Singaraja R., et al. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 1999, 23:181-192.
    • (1999) Neuron , vol.23 , pp. 181-192
    • Hodgson, J.G.1    Agopyan, N.2    Gutekunst, C.A.3    Leavitt, B.R.4    LePiane, F.5    Singaraja, R.6
  • 15
    • 0033054555 scopus 로고    scopus 로고
    • Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin
    • Schilling G., Becher M.W., Sharp A.H., Jinnah H.A., Duan K., Kotzuk J.A., et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum Mol Genet 1999, 8:397-407.
    • (1999) Hum Mol Genet , vol.8 , pp. 397-407
    • Schilling, G.1    Becher, M.W.2    Sharp, A.H.3    Jinnah, H.A.4    Duan, K.5    Kotzuk, J.A.6
  • 16
    • 0032938295 scopus 로고    scopus 로고
    • Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse
    • Wheeler V.C., Auerbach W., White J.K., Srinidhi J., Auerbach A., Ryan A., et al. Length-dependent gametic CAG repeat instability in the Huntington's disease knock-in mouse. Hum Mol Genet 1999, 8:115-122.
    • (1999) Hum Mol Genet , vol.8 , pp. 115-122
    • Wheeler, V.C.1    Auerbach, W.2    White, J.K.3    Srinidhi, J.4    Auerbach, A.5    Ryan, A.6
  • 17
    • 0032949459 scopus 로고    scopus 로고
    • A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice
    • Shelbourne P.F., Killeen N., Hevner R.F., Johnston H.M., Tecott L., Lewandoski M., et al. A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice. Hum Mol Genet 1999, 8:763-774.
    • (1999) Hum Mol Genet , vol.8 , pp. 763-774
    • Shelbourne, P.F.1    Killeen, N.2    Hevner, R.F.3    Johnston, H.M.4    Tecott, L.5    Lewandoski, M.6
  • 18
    • 0034163497 scopus 로고    scopus 로고
    • Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice
    • Wheeler V.C., White J.K., Gutekunst C.A., Vrbanac V., Weaver M., Li X.J., et al. Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice. Hum Mol Genet 2000, 9:503-513.
    • (2000) Hum Mol Genet , vol.9 , pp. 503-513
    • Wheeler, V.C.1    White, J.K.2    Gutekunst, C.A.3    Vrbanac, V.4    Weaver, M.5    Li, X.J.6
  • 19
    • 0035575858 scopus 로고    scopus 로고
    • Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease
    • Laforet G.A., Sapp E., Chase K., McIntyre C., Boyce F.M., Campbell M., et al. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci 2001, 21:9112-9123.
    • (2001) J Neurosci , vol.21 , pp. 9112-9123
    • Laforet, G.A.1    Sapp, E.2    Chase, K.3    McIntyre, C.4    Boyce, F.M.5    Campbell, M.6
  • 21
    • 16044373842 scopus 로고    scopus 로고
    • Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice
    • Mangiarini L., Sathasivam K., Seller M., Cozens B., Harper A., Hetherington C., et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996, 87:493-506.
    • (1996) Cell , vol.87 , pp. 493-506
    • Mangiarini, L.1    Sathasivam, K.2    Seller, M.3    Cozens, B.4    Harper, A.5    Hetherington, C.6
  • 22
    • 0141678246 scopus 로고    scopus 로고
    • Standardization and statistical approaches to therapeutic trials in the R6/2 mouse
    • Hockly E., Woodman B., Mahal A., Lewis C.M., Bates G. Standardization and statistical approaches to therapeutic trials in the R6/2 mouse. Brain Res Bull 2003, 61:469-479.
    • (2003) Brain Res Bull , vol.61 , pp. 469-479
    • Hockly, E.1    Woodman, B.2    Mahal, A.3    Lewis, C.M.4    Bates, G.5
  • 23
    • 60849120450 scopus 로고    scopus 로고
    • Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice
    • Morton A.J., Glynn D., Leavens W., Zheng Z., Faull R.L., Skepper J.N., et al. Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice. Neurobiol Dis 2009, 33:331-341.
    • (2009) Neurobiol Dis , vol.33 , pp. 331-341
    • Morton, A.J.1    Glynn, D.2    Leavens, W.3    Zheng, Z.4    Faull, R.L.5    Skepper, J.N.6
  • 24
    • 60849109193 scopus 로고    scopus 로고
    • CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse
    • Dragatsis I., Goldowitz D., Del Mar N., Deng Y.P., Meade C.A., Liu L., et al. CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse. Neurobiol Dis 2009, 33:315-330.
    • (2009) Neurobiol Dis , vol.33 , pp. 315-330
    • Dragatsis, I.1    Goldowitz, D.2    Del Mar, N.3    Deng, Y.P.4    Meade, C.A.5    Liu, L.6
  • 26
    • 79954630190 scopus 로고    scopus 로고
    • Gene expression profiling of R6/2 transgenic mice with different CAG repeat lengths reveals genes associated with disease onset and progression in Huntington's disease
    • Tang B., Seredenina T., Coppola G., Kuhn A., Geschwind D.H., Luthi-Carter R., et al. Gene expression profiling of R6/2 transgenic mice with different CAG repeat lengths reveals genes associated with disease onset and progression in Huntington's disease. Neurobiol Dis 2011, 42:459-467.
    • (2011) Neurobiol Dis , vol.42 , pp. 459-467
    • Tang, B.1    Seredenina, T.2    Coppola, G.3    Kuhn, A.4    Geschwind, D.H.5    Luthi-Carter, R.6
  • 27
    • 0034094873 scopus 로고    scopus 로고
    • Glutamine repeats and neurodegeneration
    • Zoghbi H.Y., Orr H.T. Glutamine repeats and neurodegeneration. Annu Rev Neurosci 2000, 23:217-247.
    • (2000) Annu Rev Neurosci , vol.23 , pp. 217-247
    • Zoghbi, H.Y.1    Orr, H.T.2
  • 28
    • 26844550967 scopus 로고    scopus 로고
    • Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease
    • Jenkins B.G., Andreassen O.A., Dedeoglu A., Leavitt B., Hayden M., Borchelt D., et al. Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease. J Neurochem 2005, 95:553-562.
    • (2005) J Neurochem , vol.95 , pp. 553-562
    • Jenkins, B.G.1    Andreassen, O.A.2    Dedeoglu, A.3    Leavitt, B.4    Hayden, M.5    Borchelt, D.6
  • 29
    • 0034889544 scopus 로고    scopus 로고
    • Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length
    • Hansson O., Castilho R.F., Korhonen L., Lindholm D., Bates G.P., Brundin P. Partial resistance to malonate-induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length. J Neurochem 2001, 78:694-703.
    • (2001) J Neurochem , vol.78 , pp. 694-703
    • Hansson, O.1    Castilho, R.F.2    Korhonen, L.3    Lindholm, D.4    Bates, G.P.5    Brundin, P.6
  • 30
    • 0034486012 scopus 로고    scopus 로고
    • Habituation of activity in an open field: a survey of inbred strains and F1 hybrids
    • Bolivar V.J., Caldarone B.J., Reilly A.A., Flaherty L. Habituation of activity in an open field: a survey of inbred strains and F1 hybrids. Behav Genet 2000, 30:285-293.
    • (2000) Behav Genet , vol.30 , pp. 285-293
    • Bolivar, V.J.1    Caldarone, B.J.2    Reilly, A.A.3    Flaherty, L.4
  • 32
    • 0032886820 scopus 로고    scopus 로고
    • Use of SHIRPA and discriminant analysis to characterise marked differences in the behavioural phenotype of six inbred mouse strains
    • Rogers D.C., Jones D.N., Nelson P.R., Jones C.M., Quilter C.A., Robinson T.L., et al. Use of SHIRPA and discriminant analysis to characterise marked differences in the behavioural phenotype of six inbred mouse strains. Behav Brain Res 1999, 105:207-217.
    • (1999) Behav Brain Res , vol.105 , pp. 207-217
    • Rogers, D.C.1    Jones, D.N.2    Nelson, P.R.3    Jones, C.M.4    Quilter, C.A.5    Robinson, T.L.6
  • 33
    • 79951567150 scopus 로고    scopus 로고
    • Modifying behavioral phenotypes in Fmr1KO mice: genetic background differences reveal autistic-like responses
    • Spencer C.M., Alekseyenko O., Hamilton S.M., Thomas A.M., Serysheva E., Yuva-Paylor L.A., et al. Modifying behavioral phenotypes in Fmr1KO mice: genetic background differences reveal autistic-like responses. Autism Res 2011, 4:40-56.
    • (2011) Autism Res , vol.4 , pp. 40-56
    • Spencer, C.M.1    Alekseyenko, O.2    Hamilton, S.M.3    Thomas, A.M.4    Serysheva, E.5    Yuva-Paylor, L.A.6
  • 34
    • 82855172160 scopus 로고    scopus 로고
    • Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability
    • doi:10.1371/journal.pone.0028409
    • Cowin RM, Bui N, Graham D, Green JR, Grueninger S, Yuva-Paylor LA, et al. Onset and progression of behavioral and molecular phenotypes in a novel congenic R6/2 line exhibiting intergenerational CAG repeat stability. PLoS ONE 6(12): e28409, doi:10.1371/journal.pone.0028409.
    • PLoS ONE , vol.6 , Issue.12
    • Cowin, R.M.1    Bui, N.2    Graham, D.3    Green, J.R.4    Grueninger, S.5    Yuva-Paylor, L.A.6
  • 35
    • 0028299278 scopus 로고
    • Characterization of benzodiazepine-sensitive behaviors in the A/J and C57BL/6J inbred strains of mice
    • Mathis C., Paul S.M., Crawley J.N. Characterization of benzodiazepine-sensitive behaviors in the A/J and C57BL/6J inbred strains of mice. Behav Genet 1994, 24:171-180.
    • (1994) Behav Genet , vol.24 , pp. 171-180
    • Mathis, C.1    Paul, S.M.2    Crawley, J.N.3
  • 36
    • 0024158498 scopus 로고
    • Thigmotaxis as a test for anxiolytic activity in rats
    • Treit D., Fundytus M. Thigmotaxis as a test for anxiolytic activity in rats. Pharmacol Biochem Behav 1988, 31:959-962.
    • (1988) Pharmacol Biochem Behav , vol.31 , pp. 959-962
    • Treit, D.1    Fundytus, M.2
  • 37
    • 0019199013 scopus 로고
    • Preliminary report of a simple animal behavior model for the anxiolytic effects of benzodiazepines
    • Crawley J., Goodwin F.K. Preliminary report of a simple animal behavior model for the anxiolytic effects of benzodiazepines. Pharmacol Biochem Behav 1980, 13:167-170.
    • (1980) Pharmacol Biochem Behav , vol.13 , pp. 167-170
    • Crawley, J.1    Goodwin, F.K.2
  • 40
    • 0037129827 scopus 로고    scopus 로고
    • Accurate normalization of real-time quantitative RT-PCR data by geometric averaging of multiple internal control genes
    • RESEARCH0034
    • Vandesompele J., De Preter K., Pattyn F., Poppe B., Van Roy N., De Paepe A., et al. Accurate normalization of real-time quantitative RT-PCR data by geometric averaging of multiple internal control genes. Genome Biol 2002, 3. RESEARCH0034.
    • (2002) Genome Biol , vol.3
    • Vandesompele, J.1    De Preter, K.2    Pattyn, F.3    Poppe, B.4    Van Roy, N.5    De Paepe, A.6
  • 41
    • 70349103737 scopus 로고    scopus 로고
    • Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease
    • Weiss A., Abramowski D., Bibel M., Bodner R., Chopra V., DiFiglia M., et al. Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease. Anal Biochem 2009, 395:8-15.
    • (2009) Anal Biochem , vol.395 , pp. 8-15
    • Weiss, A.1    Abramowski, D.2    Bibel, M.3    Bodner, R.4    Chopra, V.5    DiFiglia, M.6
  • 42
    • 17344392308 scopus 로고    scopus 로고
    • A new mathematical model for relative quantification in real-time RT-PCR
    • Pfaffl M.W. A new mathematical model for relative quantification in real-time RT-PCR. Nucleic Acids Res 2001, 29:e45.
    • (2001) Nucleic Acids Res , vol.29
    • Pfaffl, M.W.1
  • 43
    • 0036581160 scopus 로고    scopus 로고
    • Relative expression software tool (REST) for group-wise comparison and statistical analysis of relative expression results in real-time PCR
    • Pfaffl M.W., Horgan G.W., Dempfle L. Relative expression software tool (REST) for group-wise comparison and statistical analysis of relative expression results in real-time PCR. Nucleic Acids Res 2002, 30:e36.
    • (2002) Nucleic Acids Res , vol.30
    • Pfaffl, M.W.1    Horgan, G.W.2    Dempfle, L.3
  • 45
    • 0033560924 scopus 로고    scopus 로고
    • Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation
    • Carter R.J., Lione L.A., Humby T., Mangiarini L., Mahal A., Bates G.P., et al. Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation. J Neurosci 1999, 19:3248-3257.
    • (1999) J Neurosci , vol.19 , pp. 3248-3257
    • Carter, R.J.1    Lione, L.A.2    Humby, T.3    Mangiarini, L.4    Mahal, A.5    Bates, G.P.6
  • 46
    • 0031149725 scopus 로고    scopus 로고
    • High-efficiency blotting of proteins of diverse sizes following sodium dodecyl sulfate-polyacrylamide gel electrophoresis
    • Bolt M.W., Mahoney P.A. High-efficiency blotting of proteins of diverse sizes following sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Anal Biochem 1997, 247:185-192.
    • (1997) Anal Biochem , vol.247 , pp. 185-192
    • Bolt, M.W.1    Mahoney, P.A.2
  • 47
    • 18544410106 scopus 로고    scopus 로고
    • Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
    • Davies S.W., Turmaine M., Cozens B.A., DiFiglia M., Sharp A.H., Ross C.A., et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997, 90:537-548.
    • (1997) Cell , vol.90 , pp. 537-548
    • Davies, S.W.1    Turmaine, M.2    Cozens, B.A.3    DiFiglia, M.4    Sharp, A.H.5    Ross, C.A.6
  • 48
    • 0025255109 scopus 로고
    • One-dimensional gel electrophoresis
    • Academic Press, M.P. Deutscher (Ed.)
    • Garfin D.E. One-dimensional gel electrophoresis. Methods in enzymology 1990, Academic Press, p. 429. M.P. Deutscher (Ed.).
    • (1990) Methods in enzymology , pp. 429
    • Garfin, D.E.1
  • 51
    • 0032503888 scopus 로고    scopus 로고
    • Familial aggregation of schizophrenia-like symptoms in Huntington's disease
    • Tsuang D., DiGiacomo L., Lipe H., Bird T.D. Familial aggregation of schizophrenia-like symptoms in Huntington's disease. Am J Med Genet 1998, 81:323-327.
    • (1998) Am J Med Genet , vol.81 , pp. 323-327
    • Tsuang, D.1    DiGiacomo, L.2    Lipe, H.3    Bird, T.D.4
  • 52
    • 24044442568 scopus 로고    scopus 로고
    • Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing
    • Hickey M.A., Gallant K., Gross G.G., Levine M.S., Chesselet M.F. Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing. Neurobiol Dis 2005, 20:1-11.
    • (2005) Neurobiol Dis , vol.20 , pp. 1-11
    • Hickey, M.A.1    Gallant, K.2    Gross, G.G.3    Levine, M.S.4    Chesselet, M.F.5
  • 53
    • 0033500593 scopus 로고    scopus 로고
    • Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation
    • Lione L.A., Carter R.J., Hunt M.J., Bates G.P., Morton A.J., Dunnett S.B. Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation. J Neurosci 1999, 19:10428-10437.
    • (1999) J Neurosci , vol.19 , pp. 10428-10437
    • Lione, L.A.1    Carter, R.J.2    Hunt, M.J.3    Bates, G.P.4    Morton, A.J.5    Dunnett, S.B.6
  • 54
    • 0345304253 scopus 로고    scopus 로고
    • Molecular and behavioral analysis of the R6/1 Huntington's disease transgenic mouse
    • Naver B., Stub C., Moller M., Fenger K., Hansen A.K., Hasholt L., et al. Molecular and behavioral analysis of the R6/1 Huntington's disease transgenic mouse. Neuroscience 2003, 122:1049-1057.
    • (2003) Neuroscience , vol.122 , pp. 1049-1057
    • Naver, B.1    Stub, C.2    Moller, M.3    Fenger, K.4    Hansen, A.K.5    Hasholt, L.6
  • 55
    • 65249132310 scopus 로고    scopus 로고
    • Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin
    • Pouladi M.A., Graham R.K., Karasinska J.M., Xie Y., Santos R.D., Petersen A., et al. Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin. Brain 2009, 132:919-932.
    • (2009) Brain , vol.132 , pp. 919-932
    • Pouladi, M.A.1    Graham, R.K.2    Karasinska, J.M.3    Xie, Y.4    Santos, R.D.5    Petersen, A.6
  • 56
    • 17644368893 scopus 로고    scopus 로고
    • Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease
    • Van Raamsdonk J.M., Pearson J., Slow E.J., Hossain S.M., Leavitt B.R., Hayden M.R. Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J Neurosci 2005, 25:4169-4180.
    • (2005) J Neurosci , vol.25 , pp. 4169-4180
    • Van Raamsdonk, J.M.1    Pearson, J.2    Slow, E.J.3    Hossain, S.M.4    Leavitt, B.R.5    Hayden, M.R.6
  • 57
    • 0027392717 scopus 로고
    • Genetics of age-related hearing loss in mice: I. Inbred and F1 hybrid strains
    • Erway L.C., Willott J.F., Archer J.R., Harrison D.E. Genetics of age-related hearing loss in mice: I. Inbred and F1 hybrid strains. Hear Res 1993, 65:125-132.
    • (1993) Hear Res , vol.65 , pp. 125-132
    • Erway, L.C.1    Willott, J.F.2    Archer, J.R.3    Harrison, D.E.4
  • 58
    • 84860835466 scopus 로고    scopus 로고
    • Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL/6J) Huntington's disease transgenic mice
    • Brooks S., Higgs G., Janghra N., Jones L., Dunnett S.B. Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL/6J) Huntington's disease transgenic mice. Brain Res Bull 2010, 10.1016/j.brainresbull.2010.05.005.
    • (2010) Brain Res Bull
    • Brooks, S.1    Higgs, G.2    Janghra, N.3    Jones, L.4    Dunnett, S.B.5
  • 59
    • 84860840174 scopus 로고    scopus 로고
    • Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice
    • Brooks S.P., Janghra N., Workman V.L., Bayram-Weston Z., Jones L., Dunnett S.B. Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice. Brain Res Bull 2011, 10.1016/j.brainresbull.2011.01.010.
    • (2011) Brain Res Bull
    • Brooks, S.P.1    Janghra, N.2    Workman, V.L.3    Bayram-Weston, Z.4    Jones, L.5    Dunnett, S.B.6
  • 60
    • 77955466233 scopus 로고    scopus 로고
    • Environmental enrichment reduces neuronal intranuclear inclusion load but has no effect on messenger RNA expression in a mouse model of Huntington disease
    • Benn C.L., Luthi-Carter R., Kuhn A., Sadri-Vakili G., Blankson K.L., Dalai S.C., et al. Environmental enrichment reduces neuronal intranuclear inclusion load but has no effect on messenger RNA expression in a mouse model of Huntington disease. J Neuropathol Exp Neurol 2010, 69:817-827.
    • (2010) J Neuropathol Exp Neurol , vol.69 , pp. 817-827
    • Benn, C.L.1    Luthi-Carter, R.2    Kuhn, A.3    Sadri-Vakili, G.4    Blankson, K.L.5    Dalai, S.C.6
  • 61
    • 0033819849 scopus 로고    scopus 로고
    • Environmental stimulation increases survival in mice transgenic for exon 1 of the Huntington's disease gene
    • Carter R.J., Hunt M.J., Morton A.J. Environmental stimulation increases survival in mice transgenic for exon 1 of the Huntington's disease gene. Mov Disord 2000, 15:925-937.
    • (2000) Mov Disord , vol.15 , pp. 925-937
    • Carter, R.J.1    Hunt, M.J.2    Morton, A.J.3
  • 62
    • 0036152343 scopus 로고    scopus 로고
    • Environmental enrichment slows disease progression in R6/2 Huntington's disease mice
    • Hockly E., Cordery P.M., Woodman B., Mahal A., van Dellen A., Blakemore C., et al. Environmental enrichment slows disease progression in R6/2 Huntington's disease mice. Ann Neurol 2002, 51:235-242.
    • (2002) Ann Neurol , vol.51 , pp. 235-242
    • Hockly, E.1    Cordery, P.M.2    Woodman, B.3    Mahal, A.4    van Dellen, A.5    Blakemore, C.6
  • 64
    • 42549092746 scopus 로고    scopus 로고
    • Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease
    • van Dellen A., Cordery P.M., Spires T.L., Blakemore C., Hannan A.J. Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease. BMC Neurosci 2008, 9:34.
    • (2008) BMC Neurosci , vol.9 , pp. 34
    • van Dellen, A.1    Cordery, P.M.2    Spires, T.L.3    Blakemore, C.4    Hannan, A.J.5
  • 65
    • 79953034289 scopus 로고    scopus 로고
    • Evaluating gene expression in C57BL/6J and DBA/2J mouse striatum using RNA-Seq and microarrays
    • Bottomly D., Walter N.A., Hunter J.E., Darakjian P., Kawane S., Buck K.J., et al. Evaluating gene expression in C57BL/6J and DBA/2J mouse striatum using RNA-Seq and microarrays. PLoS ONE 2011, 6:e17820.
    • (2011) PLoS ONE , vol.6
    • Bottomly, D.1    Walter, N.A.2    Hunter, J.E.3    Darakjian, P.4    Kawane, S.5    Buck, K.J.6
  • 67
    • 33748123054 scopus 로고    scopus 로고
    • Gene expression profiling in the striatum of inbred mouse strains with distinct opioid-related phenotypes
    • Korostynski M., Kaminska-Chowaniec D., Piechota M., Przewlocki R. Gene expression profiling in the striatum of inbred mouse strains with distinct opioid-related phenotypes. BMC Genomics 2006, 7:146.
    • (2006) BMC Genomics , vol.7 , pp. 146
    • Korostynski, M.1    Kaminska-Chowaniec, D.2    Piechota, M.3    Przewlocki, R.4
  • 68
    • 0032101287 scopus 로고    scopus 로고
    • The influence of huntingtin protein size on nuclear localization and cellular toxicity
    • Hackam A.S., Singaraja R., Wellington C.L., Metzler M., McCutcheon K., Zhang T., et al. The influence of huntingtin protein size on nuclear localization and cellular toxicity. J Cell Biol 1998, 141:1097-1105.
    • (1998) J Cell Biol , vol.141 , pp. 1097-1105
    • Hackam, A.S.1    Singaraja, R.2    Wellington, C.L.3    Metzler, M.4    McCutcheon, K.5    Zhang, T.6
  • 69
    • 0029984570 scopus 로고    scopus 로고
    • Nucleocytoplasmic transport
    • Gorlich D., Mattaj I.W. Nucleocytoplasmic transport. Science 1996, 271:1513-1518.
    • (1996) Science , vol.271 , pp. 1513-1518
    • Gorlich, D.1    Mattaj, I.W.2
  • 70
    • 0037604556 scopus 로고    scopus 로고
    • Peering through the pore: nuclear pore complex structure, assembly, and function
    • Suntharalingam M., Wente S.R. Peering through the pore: nuclear pore complex structure, assembly, and function. Dev Cell 2003, 4:775-789.
    • (2003) Dev Cell , vol.4 , pp. 775-789
    • Suntharalingam, M.1    Wente, S.R.2
  • 71
    • 36749081534 scopus 로고    scopus 로고
    • Crossing the nuclear envelope: hierarchical regulation of nucleocytoplasmic transport
    • Terry L.J., Shows E.B., Wente S.R. Crossing the nuclear envelope: hierarchical regulation of nucleocytoplasmic transport. Science 2007, 318:1412-1416.
    • (2007) Science , vol.318 , pp. 1412-1416
    • Terry, L.J.1    Shows, E.B.2    Wente, S.R.3
  • 72
    • 27544477225 scopus 로고    scopus 로고
    • Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease
    • Benn C.L., Landles C., Li H., Strand A.D., Woodman B., Sathasivam K., et al. Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease. Hum Mol Genet 2005, 14:3065-3078.
    • (2005) Hum Mol Genet , vol.14 , pp. 3065-3078
    • Benn, C.L.1    Landles, C.2    Li, H.3    Strand, A.D.4    Woodman, B.5    Sathasivam, K.6
  • 73
    • 33749176269 scopus 로고    scopus 로고
    • Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state
    • Kitamura A., Kubota H., Pack C.G., Matsumoto G., Hirayama S., Takahashi Y., et al. Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state. Nat Cell Biol 2006, 8:1163-1170.
    • (2006) Nat Cell Biol , vol.8 , pp. 1163-1170
    • Kitamura, A.1    Kubota, H.2    Pack, C.G.3    Matsumoto, G.4    Hirayama, S.5    Takahashi, Y.6
  • 74
    • 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
  • 75
    • 44649113841 scopus 로고    scopus 로고
    • Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin
    • Wang C.E., Zhou H., McGuire J.R., Cerullo V., Lee B., Li S.H., et al. Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J Cell Biol 2008, 181:803-816.
    • (2008) J Cell Biol , vol.181 , pp. 803-816
    • Wang, C.E.1    Zhou, H.2    McGuire, J.R.3    Cerullo, V.4    Lee, B.5    Li, S.H.6
  • 76
    • 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., et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997, 277:1990-1993.
    • (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
  • 77
    • 0038104366 scopus 로고    scopus 로고
    • Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context
    • Jackson W.S., Tallaksen-Greene S.J., Albin R.L., Detloff P.J. Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context. Hum Mol Genet 2003, 12:1621-1629.
    • (2003) Hum Mol Genet , vol.12 , pp. 1621-1629
    • Jackson, W.S.1    Tallaksen-Greene, S.J.2    Albin, R.L.3    Detloff, P.J.4
  • 78
    • 0032811511 scopus 로고    scopus 로고
    • Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice
    • Li H., Li S.H., Cheng A.L., Mangiarini L., Bates G.P., Li X.J. Ultrastructural localization and progressive formation of neuropil aggregates in Huntington's disease transgenic mice. Hum Mol Genet 1999, 8:1227-1236.
    • (1999) Hum Mol Genet , vol.8 , pp. 1227-1236
    • Li, H.1    Li, S.H.2    Cheng, A.L.3    Mangiarini, L.4    Bates, G.P.5    Li, X.J.6
  • 80
    • 0036850529 scopus 로고    scopus 로고
    • Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells
    • Yang W., Dunlap J.R., Andrews R.B., Wetzel R. Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells. Hum Mol Genet 2002, 11:2905-2917.
    • (2002) Hum Mol Genet , vol.11 , pp. 2905-2917
    • Yang, W.1    Dunlap, J.R.2    Andrews, R.B.3    Wetzel, R.4
  • 81
    • 23844525922 scopus 로고    scopus 로고
    • Huntington's disease genetics
    • Myers R.H. Huntington's disease genetics. NeuroRx 2004, 1:255-262.
    • (2004) NeuroRx , vol.1 , pp. 255-262
    • Myers, R.H.1
  • 82
    • 0033556344 scopus 로고    scopus 로고
    • Analysis of a very large trinucleotide repeat in a patient with juvenile Huntington's disease
    • Nance M.A., Mathias-Hagen V., Breningstall G., Wick M.J., McGlennen R.C. Analysis of a very large trinucleotide repeat in a patient with juvenile Huntington's disease. Neurology 1999, 52:392-394.
    • (1999) Neurology , vol.52 , pp. 392-394
    • Nance, M.A.1    Mathias-Hagen, V.2    Breningstall, G.3    Wick, M.J.4    McGlennen, R.C.5
  • 83
    • 79953328501 scopus 로고    scopus 로고
    • Juvenile Huntington disease in an 18-month-old boy revealed by global developmental delay and reduced cerebellar volume
    • Nicolas G., Devys D., Goldenberg A., Maltete D., Herve C., Hannequin D., et al. Juvenile Huntington disease in an 18-month-old boy revealed by global developmental delay and reduced cerebellar volume. Am J Med Genet A 2011, 155A:815-818.
    • (2011) Am J Med Genet A , vol.155 A , pp. 815-818
    • Nicolas, G.1    Devys, D.2    Goldenberg, A.3    Maltete, D.4    Herve, C.5    Hannequin, D.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.