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Volumn 88, Issue 2-3, 2012, Pages 104-112

Light and electron microscopic characterization of the evolution of cellular pathology in the R6/1 Huntington's disease transgenic mice

Author keywords

Aggregations; Huntington's disease; Inclusions; R6 1; Transgenic mice; Transmission electron microscope (TEM)

Indexed keywords

GLIAL FIBRILLARY ACIDIC PROTEIN;

EID: 84860837080     PISSN: 03619230     EISSN: 18732747     Source Type: Journal    
DOI: 10.1016/j.brainresbull.2011.07.009     Document Type: Article
Times cited : (27)

References (84)
  • 1
    • 84914639435 scopus 로고
    • Estimation of nuclear population from microtome sections
    • Abercrombie M. Estimation of nuclear population from microtome sections. Anat. Rec. 1946, 239-247.
    • (1946) Anat. Rec. , pp. 239-247
    • Abercrombie, M.1
  • 2
    • 7244236320 scopus 로고    scopus 로고
    • Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death
    • Arrasate M., Mitra S., Schweitzer E.S., Segal M.R., Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004, 431:805-810.
    • (2004) Nature , vol.431 , pp. 805-810
    • Arrasate, M.1    Mitra, S.2    Schweitzer, E.S.3    Segal, M.R.4    Finkbeiner, S.5
  • 3
    • 0013218415 scopus 로고    scopus 로고
    • Oxford University Press, G.P. Bates, P.S. Harper, L. Jones (Eds.)
    • Bates G.P., Harper P.S., Jones L. Huntington's Disease 2002, Oxford University Press. G.P. Bates, P.S. Harper, L. Jones (Eds.).
    • (2002) Huntington's Disease
    • Bates, G.P.1    Harper, P.S.2    Jones, L.3
  • 6
    • 84860836853 scopus 로고    scopus 로고
    • Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 transgenic mice
    • Bayram-Weston Z., Jones L., Dunnett S.B., Brooks S.P. Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 transgenic mice. Brain Res. Bull. 2012, 88:137-147.
    • (2012) Brain Res. Bull. , vol.88 , pp. 137-147
    • Bayram-Weston, Z.1    Jones, L.2    Dunnett, S.B.3    Brooks, S.P.4
  • 7
    • 84860841633 scopus 로고    scopus 로고
    • Light and electron microscopic characterization of the evolution of cellular pathology in the Hdh(CAG)150 Huntington's disease knock-in mouse
    • Bayram-Weston Z., Torres E.M., Jones L., Dunnett S.B., Brooks S.P. Light and electron microscopic characterization of the evolution of cellular pathology in the Hdh(CAG)150 Huntington's disease knock-in mouse. Brain Res. Bull. 2012, 88:189-198.
    • (2012) Brain Res. Bull. , vol.88 , pp. 189-198
    • Bayram-Weston, Z.1    Torres, E.M.2    Jones, L.3    Dunnett, S.B.4    Brooks, S.P.5
  • 8
    • 1642418261 scopus 로고    scopus 로고
    • Early exploratory behavior abnormalities in R6/1 Huntington's disease transgenic mice
    • Bolivar V.J., Manley K., Messer A. Early exploratory behavior abnormalities in R6/1 Huntington's disease transgenic mice. Brain Res. 2004, 1005:29-35.
    • (2004) Brain Res. , vol.1005 , pp. 29-35
    • Bolivar, V.J.1    Manley, K.2    Messer, A.3
  • 9
    • 33845336461 scopus 로고    scopus 로고
    • Assessing autism-like behavior in mice: variations in social interactions among inbred strains
    • Bolivar V.J., Walters S.R., Phoenix J.L. Assessing autism-like behavior in mice: variations in social interactions among inbred strains. Behav. Brain Res. 2007, 176:21-26.
    • (2007) Behav. Brain Res. , vol.176 , pp. 21-26
    • Bolivar, V.J.1    Walters, S.R.2    Phoenix, J.L.3
  • 10
    • 0027252529 scopus 로고
    • Apoptosis or programmed cell death?
    • Bowen I.D. Apoptosis or programmed cell death?. Cell Biol. Int. 1993, 17:365-380.
    • (1993) Cell Biol. Int. , vol.17 , pp. 365-380
    • Bowen, I.D.1
  • 11
    • 33750292972 scopus 로고    scopus 로고
    • Cell death in the nervous system
    • Bredesen D.E., Rao R.V., Mehlen P. Cell death in the nervous system. Nature 2006, 443:796-802.
    • (2006) Nature , vol.443 , pp. 796-802
    • Bredesen, D.E.1    Rao, R.V.2    Mehlen, P.3
  • 12
    • 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
  • 16
    • 84860838730 scopus 로고    scopus 로고
    • Proteomic changes in the brains of Huntington's disease mouse models reflect pathology and implicate mitochondrial changes
    • Deschepper M., Hoogendoorn B., Brooks S., Dunnett S.B., Jones L. Proteomic changes in the brains of Huntington's disease mouse models reflect pathology and implicate mitochondrial changes. Brain Res. Bull. 2011, 10.1016/j.brainresbull.2011.01.012.
    • (2011) Brain Res. Bull.
    • Deschepper, M.1    Hoogendoorn, B.2    Brooks, S.3    Dunnett, S.B.4    Jones, L.5
  • 17
    • 0030752709 scopus 로고    scopus 로고
    • Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
    • DiFiglia M., Sapp E., Chase K.O., Davies S.W., Bates G.P., Vonsattel J.P., Aronin N. 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    Aronin, N.7
  • 18
    • 0030320779 scopus 로고    scopus 로고
    • Immunohistochemical findings in Huntington's Chorea: report of 9 cases
    • Galatioto S. Immunohistochemical findings in Huntington's Chorea: report of 9 cases. Pathologica 1996, 88:491-499.
    • (1996) Pathologica , vol.88 , pp. 491-499
    • Galatioto, S.1
  • 19
    • 0033457954 scopus 로고    scopus 로고
    • Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease
    • Georgiou N., Bradshaw J.L., Chiu E., Tudor A., O'Gorman L., Phillips J.G. Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease. Mov. Disord. 1999, 14:320-325.
    • (1999) Mov. Disord. , vol.14 , pp. 320-325
    • Georgiou, N.1    Bradshaw, J.L.2    Chiu, E.3    Tudor, A.4    O'Gorman, L.5    Phillips, J.G.6
  • 20
    • 57249084239 scopus 로고    scopus 로고
    • Neuronal cell death in neurodegenerative diseases: recurring themes around protein handling
    • Gorman A.M. Neuronal cell death in neurodegenerative diseases: recurring themes around protein handling. J. Cell Mol. Med. 2008, 12:2263-2280.
    • (2008) J. Cell Mol. Med. , vol.12 , pp. 2263-2280
    • Gorman, A.M.1
  • 22
    • 0021982117 scopus 로고
    • Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease
    • Graveland G.A., Williams R.S., DiFiglia M. Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science 1985, 227:770-773.
    • (1985) Science , vol.227 , pp. 770-773
    • Graveland, G.A.1    Williams, R.S.2    DiFiglia, M.3
  • 24
    • 77951252744 scopus 로고    scopus 로고
    • Huntington's disease: the case for genetic modifiers
    • Gusella J.F., MacDonald M.E. Huntington's disease: the case for genetic modifiers. Genome Med. 2009, 1:80.
    • (2009) Genome Med. , vol.1 , pp. 80
    • Gusella, J.F.1    MacDonald, M.E.2
  • 26
    • 0035783462 scopus 로고    scopus 로고
    • Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene
    • Hansson O., Guatteo E., Mercuri N.B., Bernardi G., Li X.J., Castilho R.F., Brundin P. Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene. Eur. J. Neurosci. 2001, 14:1492-1504.
    • (2001) Eur. J. Neurosci. , vol.14 , pp. 1492-1504
    • Hansson, O.1    Guatteo, E.2    Mercuri, N.B.3    Bernardi, G.4    Li, X.J.5    Castilho, R.F.6    Brundin, P.7
  • 28
    • 58149457367 scopus 로고    scopus 로고
    • Huntington disease in subjects from an Israeli Karaite community carrying alleles of intermediate and expanded CAG repeats in the HTT gene: Huntington disease or phenocopy?
    • Herishanu Y.O., Parvari R., Pollack Y., Shelef I., Marom B., Martino T., Cannella M., Squitieri F. Huntington disease in subjects from an Israeli Karaite community carrying alleles of intermediate and expanded CAG repeats in the HTT gene: Huntington disease or phenocopy?. J. Neurol. Sci. 2009, 277:143-146.
    • (2009) J. Neurol. Sci. , vol.277 , pp. 143-146
    • Herishanu, Y.O.1    Parvari, R.2    Pollack, Y.3    Shelef, I.4    Marom, B.5    Martino, T.6    Cannella, M.7    Squitieri, F.8
  • 31
    • 41749121925 scopus 로고    scopus 로고
    • Brain gene expression correlates with changes in behavior in the R6/1 mouse model of Huntington's disease
    • Hodges A., Hughes G., Brooks S., Elliston L., Holmans P., Dunnett S.B., Jones L. Brain gene expression correlates with changes in behavior in the R6/1 mouse model of Huntington's disease. Genes Brain Behav. 2008, 7:288-299.
    • (2008) Genes Brain Behav. , vol.7 , pp. 288-299
    • Hodges, A.1    Hughes, G.2    Brooks, S.3    Elliston, L.4    Holmans, P.5    Dunnett, S.B.6    Jones, L.7
  • 32
    • 0021139033 scopus 로고
    • Reduced Purkinje cell density in Huntington's disease
    • Jeste D.V., Barban L., Parisi J. Reduced Purkinje cell density in Huntington's disease. Exp. Neurol. 1984, 85:78-86.
    • (1984) Exp. Neurol. , vol.85 , pp. 78-86
    • Jeste, D.V.1    Barban, L.2    Parisi, J.3
  • 33
    • 77956801270 scopus 로고
    • Anatomical methods in cell death
    • Academic Press Inc., San Diego, L.M. Schwartz, B.A. Osborno (Eds.)
    • Kerr J.F.R., Gobe G.G., Winterford C.M., Harmon B.V. Anatomical methods in cell death. Methods in Cell Biology 1995, 1-27. Academic Press Inc., San Diego. L.M. Schwartz, B.A. Osborno (Eds.).
    • (1995) Methods in Cell Biology , pp. 1-27
    • Kerr, J.F.R.1    Gobe, G.G.2    Winterford, C.M.3    Harmon, B.V.4
  • 34
    • 0015383455 scopus 로고
    • Apoptosis: a basic biological phenomenon with wide-ranging implications in tissue kinetics
    • Kerr J.F., Wyllie A.H., Currie A.R. Apoptosis: a basic biological phenomenon with wide-ranging implications in tissue kinetics. Br. J. Cancer 1972, 26:239-257.
    • (1972) Br. J. Cancer , vol.26 , pp. 239-257
    • Kerr, J.F.1    Wyllie, A.H.2    Currie, A.R.3
  • 36
    • 57049117856 scopus 로고    scopus 로고
    • Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities
    • Kim I., Xu W., Reed J.C. Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities. Nat. Rev. Drug Discov. 2008, 7:1013-1030.
    • (2008) Nat. Rev. Drug Discov. , vol.7 , pp. 1013-1030
    • Kim, I.1    Xu, W.2    Reed, J.C.3
  • 41
    • 4344669917 scopus 로고    scopus 로고
    • Cognitive changes in patients with Huntington's disease (HD) and asymptomatic carriers of the HD mutation-a longitudinal follow-up study
    • Lemiere J., Decruyenaere M., Evers-Kiebooms G., Vandenbussche E., Dom R. Cognitive changes in patients with Huntington's disease (HD) and asymptomatic carriers of the HD mutation-a longitudinal follow-up study. J. Neurol. 2004, 251:935-942.
    • (2004) J. Neurol. , vol.251 , pp. 935-942
    • Lemiere, J.1    Decruyenaere, M.2    Evers-Kiebooms, G.3    Vandenbussche, E.4    Dom, R.5
  • 42
    • 0042921188 scopus 로고    scopus 로고
    • Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release
    • Li H., Wyman T., Yu Z.X., Li S.H., Li X.J. Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release. Hum. Mol. Genet. 2003, 12:2021-2030.
    • (2003) Hum. Mol. Genet. , vol.12 , pp. 2021-2030
    • Li, H.1    Wyman, T.2    Yu, Z.X.3    Li, S.H.4    Li, X.J.5
  • 44
    • 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
  • 48
    • 0028891783 scopus 로고
    • Apoptosis, oncosis, and necrosis: an overview of cell death
    • Majno G., Joris I. Apoptosis, oncosis, and necrosis: an overview of cell death. Am. J. Pathol. 1995, 146:3-15.
    • (1995) Am. J. Pathol. , vol.146 , pp. 3-15
    • Majno, G.1    Joris, I.2
  • 49
    • 0031056685 scopus 로고    scopus 로고
    • Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation
    • Mangiarini L., Sathasivam K., Mahal A., Mott R., Seller M., Bates G.P. Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation. Nat. Genet. 1997, 15:197-200.
    • (1997) Nat. Genet. , vol.15 , pp. 197-200
    • Mangiarini, L.1    Sathasivam, K.2    Mahal, A.3    Mott, R.4    Seller, M.5    Bates, G.P.6
  • 52
    • 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. J. Neurosci. 2002, 22:8266-8276.
    • (2002) J. Neurosci. , vol.22 , 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
  • 53
    • 77955359169 scopus 로고    scopus 로고
    • Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis
    • Miller J., Arrasate M., Shaby B.A., Mitra S., Masliah E., Finkbeiner S. Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis. J. Neurosci. 2010, 30:10541-10550.
    • (2010) J. Neurosci. , vol.30 , pp. 10541-10550
    • Miller, J.1    Arrasate, M.2    Shaby, B.A.3    Mitra, S.4    Masliah, E.5    Finkbeiner, S.6
  • 55
  • 57
    • 39249084983 scopus 로고    scopus 로고
    • Gene-environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington's disease transgenic mice
    • Nithianantharajah J., Barkus C., Murphy M., Hannan A.J. Gene-environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington's disease transgenic mice. Neurobiol. Dis. 2008, 29:490-504.
    • (2008) Neurobiol. Dis. , vol.29 , pp. 490-504
    • Nithianantharajah, J.1    Barkus, C.2    Murphy, M.3    Hannan, A.J.4
  • 59
    • 0036254430 scopus 로고    scopus 로고
    • Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease
    • Petersen A., Chase K., Puschban Z., DiFiglia M., Brundin P., Aronin N. Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease. Exp. Neurol. 2002, 175:297-300.
    • (2002) Exp. Neurol. , vol.175 , pp. 297-300
    • Petersen, A.1    Chase, K.2    Puschban, Z.3    DiFiglia, M.4    Brundin, P.5    Aronin, N.6
  • 63
    • 0019455846 scopus 로고
    • Cerebellar atrophy in Huntington's disease
    • Rodda R.A. Cerebellar atrophy in Huntington's disease. J. Neurol. Sci. 1981, 50:147-157.
    • (1981) J. Neurol. Sci. , vol.50 , pp. 147-157
    • Rodda, R.A.1
  • 65
    • 0031446233 scopus 로고    scopus 로고
    • Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?
    • Ross C.A. Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?. Neuron 1997, 19:1147-1150.
    • (1997) Neuron , vol.19 , pp. 1147-1150
    • Ross, C.A.1
  • 66
    • 0030741184 scopus 로고    scopus 로고
    • Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology
    • Ross C.A., Becher M.W., Colomer V., Engelender S., Wood J.D., Sharp A.H. Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology. Brain Pathol. 1997, 7:1003-1016.
    • (1997) Brain Pathol. , vol.7 , pp. 1003-1016
    • Ross, C.A.1    Becher, M.W.2    Colomer, V.3    Engelender, S.4    Wood, J.D.5    Sharp, A.H.6
  • 67
    • 33750363298 scopus 로고    scopus 로고
    • The roles of intracellular protein-degradation pathways in neurodegeneration
    • Rubinsztein D.C. The roles of intracellular protein-degradation pathways in neurodegeneration. Nature 2006, 443:780-786.
    • (2006) Nature , vol.443 , pp. 780-786
    • Rubinsztein, D.C.1
  • 69
    • 0032904145 scopus 로고    scopus 로고
    • Intracellular inclusions pathological markers in diseases caused by expanded polyglutamine tracts?
    • Rubinsztein D.C., Wyttenbach A., Rankin J. Intracellular inclusions pathological markers in diseases caused by expanded polyglutamine tracts?. J. Med. Genet. 1999, 36:265-270.
    • (1999) J. Med. Genet. , vol.36 , pp. 265-270
    • Rubinsztein, D.C.1    Wyttenbach, A.2    Rankin, J.3
  • 70
    • 33747378753 scopus 로고    scopus 로고
    • Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat
    • Ruocco H.H., Lopes-Cendes I., Li L.M., Santos-Silva M., Cendes F. Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat. Braz. J. Med. Biol. Res. 2006, 39:1129-1136.
    • (2006) Braz. J. Med. Biol. Res. , vol.39 , pp. 1129-1136
    • Ruocco, H.H.1    Lopes-Cendes, I.2    Li, L.M.3    Santos-Silva, M.4    Cendes, F.5
  • 72
    • 0032475931 scopus 로고    scopus 로고
    • Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions
    • Saudou F., Finkbeiner S., Devys D., Greenberg M.E. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 1998, 95:55-66.
    • (1998) Cell , vol.95 , pp. 55-66
    • Saudou, F.1    Finkbeiner, S.2    Devys, D.3    Greenberg, M.E.4
  • 73
    • 79952585486 scopus 로고    scopus 로고
    • Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage
    • Shirendeb U., Reddy A.P., Manczak M., Calkins M.J., Mao P., Tagle D.A., Hemachandra R.P. Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage. Hum. Mol. Genet. 2011, 20:1438-1455.
    • (2011) Hum. Mol. Genet. , vol.20 , pp. 1438-1455
    • Shirendeb, U.1    Reddy, A.P.2    Manczak, M.3    Calkins, M.J.4    Mao, P.5    Tagle, D.A.6    Hemachandra, R.P.7
  • 74
    • 0032475877 scopus 로고    scopus 로고
    • Nuclear inclusions in glutamine repeat disorders: are they pernicious coincidental, or beneficial?
    • Sisodia S.S. Nuclear inclusions in glutamine repeat disorders: are they pernicious coincidental, or beneficial?. Cell 1998, 95:1-4.
    • (1998) Cell , vol.95 , pp. 1-4
    • Sisodia, S.S.1
  • 76
    • 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. Cell 1993, 72:971-983. The Huntington's Disease Collaborative Research Group.
    • (1993) Cell , vol.72 , pp. 971-983
  • 78
    • 33847270651 scopus 로고    scopus 로고
    • Reduced expression of PSA-NCAM in the hippocampus and piriform cortex of the R6/1 and R6/2 mouse models of Huntington's disease
    • van der Borght K., Brundin P. Reduced expression of PSA-NCAM in the hippocampus and piriform cortex of the R6/1 and R6/2 mouse models of Huntington's disease. Exp. Neurol. 2007, 204:473-478.
    • (2007) Exp. Neurol. , vol.204 , pp. 473-478
    • van der Borght, K.1    Brundin, P.2
  • 79
    • 33947286031 scopus 로고    scopus 로고
    • Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain
    • Van Raamsdonk J.M., Metzler M., Slow E., Pearson J., Schwab C., Carroll J., Graham R.K., Leavitt B.R., Hayden M.R. Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain. Neurobiol. Dis. 2007, 26:189-200.
    • (2007) Neurobiol. Dis. , vol.26 , pp. 189-200
    • Van Raamsdonk, J.M.1    Metzler, M.2    Slow, E.3    Pearson, J.4    Schwab, C.5    Carroll, J.6    Graham, R.K.7    Leavitt, B.R.8    Hayden, M.R.9
  • 80
    • 29644433445 scopus 로고    scopus 로고
    • Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease
    • Van Raamsdonk J.M., Murphy Z., Slow E.J., Leavitt B.R., Hayden M.R. Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 2005, 14:3823-3835.
    • (2005) Hum. Mol. Genet. , vol.14 , pp. 3823-3835
    • Van Raamsdonk, J.M.1    Murphy, Z.2    Slow, E.J.3    Leavitt, B.R.4    Hayden, M.R.5
  • 81
    • 0038205745 scopus 로고    scopus 로고
    • Targeting programmed cell death in neurodegenerative diseases
    • Vila M., Przedborski S. Targeting programmed cell death in neurodegenerative diseases. Nat. Rev. Neurosci. 2003, 4:365-375.
    • (2003) Nat. Rev. Neurosci. , vol.4 , pp. 365-375
    • Vila, M.1    Przedborski, S.2
  • 84
    • 0034737299 scopus 로고    scopus 로고
    • Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease
    • Yamamoto A., Lucas J.J., Hen R. Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 2000, 101:57-66.
    • (2000) Cell , vol.101 , pp. 57-66
    • Yamamoto, A.1    Lucas, J.J.2    Hen, R.3


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