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Volumn 19, Issue 8, 2013, Pages 1030-1038

Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models

(18)  Marco, Sonia a   Giralt, Albert b,c   Petrovic, Milos M a,d   Pouladi, Mahmoud A e,f   Martínez Turrillas, Rebeca a   Martínez Hernández, José g   Kaltenbach, Linda S h   Torres Peraza, Jesús b   Graham, Rona K e   Watanabe, Masahiko i   Luján, Rafael g   Nakanishi, Nobuki j   Lipton, Stuart A j   Lo, Donald C h   Hayden, Michael R e,f,k   Alberch, Jordi b   Wesseling, John F a   Pérez Otaño, Isabel a  


Author keywords

[No Author keywords available]

Indexed keywords

GLUN3A PROTEIN; HUNTINGTIN; MUTANT PROTEIN; N METHYL DEXTRO ASPARTIC ACID RECEPTOR; PROTEIN; UNCLASSIFIED DRUG;

EID: 84882275870     PISSN: 10788956     EISSN: 1546170X     Source Type: Journal    
DOI: 10.1038/nm.3246     Document Type: Article
Times cited : (90)

References (59)
  • 1
    • 0030752709 scopus 로고    scopus 로고
    • Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
    • DiFiglia, M. et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990-1993 (1997).
    • (1997) Science , vol.277 , pp. 1990-1993
    • Difiglia, M.1
  • 2
    • 0028989602 scopus 로고
    • Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
    • DiFiglia, M. et al. Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 14, 1075-1081 (1995).
    • (1995) Neuron , vol.14 , pp. 1075-1081
    • Difiglia, M.1
  • 3
    • 3042717240 scopus 로고    scopus 로고
    • Cellular toxicity of polyglutamine expansion proteins: Mechanism of transcription factor deactivation
    • Schaffar, G. et al. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol. Cell 15, 95-105 (2004).
    • (2004) Mol. Cell , vol.15 , pp. 95-105
    • Schaffar, G.1
  • 4
    • 34249715853 scopus 로고    scopus 로고
    • Huntingtin interacting proteins are genetic modifiers of neurodegeneration
    • Kaltenbach, L.S. et al. Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet. 3, e82 (2007).
    • (2007) PLoS Genet , vol.3
    • Kaltenbach, L.S.1
  • 5
    • 4644231870 scopus 로고    scopus 로고
    • A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease
    • Goehler, H. et al. A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington's disease. Mol. Cell 15, 853-865 (2004).
    • (2004) Mol. Cell , vol.15 , pp. 853-865
    • Goehler, H.1
  • 6
    • 0842322740 scopus 로고    scopus 로고
    • Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction
    • Qin, Z.H. et al. Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. J. Neurosci. 24, 269-281 (2004).
    • (2004) J. Neurosci , vol.24 , pp. 269-281
    • Qin, Z.H.1
  • 7
    • 0142184100 scopus 로고    scopus 로고
    • Huntington's disease: A synaptopathy?
    • Li, J.Y., Plomann, M. & Brundin, P. Huntington's disease: a synaptopathy? Trends Mol. Med. 9, 414-420 (2003).
    • (2003) Trends Mol. Med , vol.9 , pp. 414-420
    • Li, J.Y.1    Plomann, M.2    Brundin, P.3
  • 8
    • 33745751770 scopus 로고    scopus 로고
    • Endocytosis and synaptic removal of nr3a-containing nmda receptors by pacsin1/syndapin1
    • Pérez-Otaño, I. et al. Endocytosis and synaptic removal of NR3A-containing NMDA receptors by PACSIN1/syndapin1. Nat. Neurosci. 9, 611-621 (2006).
    • (2006) Nat. Neurosci , vol.9 , pp. 611-621
    • Pérez-Otaño, I.1
  • 9
    • 0033571743 scopus 로고    scopus 로고
    • Enhanced sensitivity to N-methyl-d-aspartate receptor activation in transgenic and knock-in mouse models of Huntington's disease
    • Levine, M.S. et al. Enhanced sensitivity to N-methyl-d-aspartate receptor activation in transgenic and knock-in mouse models of Huntington's disease. J. Neurosci. Res. 58, 515-532 (1999).
    • (1999) J. Neurosci. Res , vol.58 , pp. 515-532
    • Levine, M.S.1
  • 10
    • 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. et al. Increased sensitivity to N-methyl-d-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington's disease. Neuron 33, 849-860 (2002).
    • (2002) Neuron , vol.33 , pp. 849-860
    • Zeron, M.M.1
  • 11
    • 0035889535 scopus 로고    scopus 로고
    • NMDA receptor function in mouse models of Huntington disease
    • Cepeda, C. et al. NMDA receptor function in mouse models of Huntington disease. J. Neurosci. Res. 66, 525-539 (2001).
    • (2001) J. Neurosci. Res , vol.66 , pp. 525-539
    • Cepeda, C.1
  • 12
    • 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. et al. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J. Neurosci. 21, 9112-9123 (2001).
    • (2001) J. Neurosci , vol.21 , pp. 9112-9123
    • Laforet, G.A.1
  • 13
    • 74549181538 scopus 로고    scopus 로고
    • Early increase in extrasynaptic nmda receptor signaling and expression contributes to phenotype onset in huntington's disease mice
    • Milnerwood, A.J. et al. Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice. Neuron 65, 178-190 (2010).
    • (2010) Neuron , vol.65 , pp. 178-190
    • Milnerwood, A.J.1
  • 14
    • 0022446150 scopus 로고
    • Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid
    • Beal, M.F. et al. Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid. Nature 321, 168-171 (1986).
    • (1986) Nature , vol.321 , pp. 168-171
    • Beal, M.F.1
  • 15
    • 0032575065 scopus 로고    scopus 로고
    • Increased nmda current and spine density in mice lacking the nmda receptor subunit nr3a
    • Das, S. et al. Increased NMDA current and spine density in mice lacking the NMDA receptor subunit NR3A. Nature 393, 377-381 (1998).
    • (1998) Nature , vol.393 , pp. 377-381
    • Das, S.1
  • 16
    • 68249153704 scopus 로고    scopus 로고
    • Downregulation of nr3a-containing nmdars is required for synapse maturation and memory consolidation
    • Roberts, A.C. et al. Downregulation of NR3A-containing NMDARs is required for synapse maturation and memory consolidation. Neuron 63, 342-356 (2009).
    • (2009) Neuron , vol.63 , pp. 342-356
    • Roberts, A.C.1
  • 17
    • 0036796261 scopus 로고    scopus 로고
    • PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
    • Modregger, J., DiProspero, N.A., Charles, V., Tagle, D.A. & Plomann, M. PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains. Hum. Mol. Genet. 11, 2547-2558 (2002).
    • (2002) Hum. Mol. Genet , vol.11 , pp. 2547-2558
    • Modregger, J.1    Diprospero, N.A.2    Charles, V.3    Tagle, D.A.4    Plomann, M.5
  • 18
    • 42049086100 scopus 로고    scopus 로고
    • Opposing effects of polyglutamine expansion on native protein complexes contribute to sca1
    • Lim, J. et al. Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. Nature 452, 713-718 (2008).
    • (2008) Nature , vol.452 , pp. 713-718
    • Lim, J.1
  • 19
    • 10744227174 scopus 로고    scopus 로고
    • Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
    • Slow, E.J. et al. Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease. Hum. Mol. Genet. 12, 1555-1567 (2003).
    • (2003) Hum. Mol. Genet , vol.12 , pp. 1555-1567
    • Slow, E.J.1
  • 20
    • 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 431, 805-810 (2004).
    • (2004) Nature , vol.431 , pp. 805-810
    • Arrasate, M.1    Mitra, S.2    Schweitzer, E.S.3    Segal, M.R.4    Finkbeiner, S.5
  • 21
    • 2142765951 scopus 로고    scopus 로고
    • A syntaxin 1 Gα(o) and N-type calcium channel complex at a presynaptic nerve terminal: Analysis by quantitative immunocolocalization
    • Li, Q. et al. A syntaxin 1, Gα(o), and N-type calcium channel complex at a presynaptic nerve terminal: analysis by quantitative immunocolocalization. J. Neurosci. 24, 4070-4081 (2004).
    • (2004) J. Neurosci , vol.24 , pp. 4070-4081
    • Li, Q.1
  • 22
    • 0035866095 scopus 로고    scopus 로고
    • Assembly with the NR1 subunit is required for surface expression of NR3A-containing nmda receptors
    • Perez-Otaño, I. et al. Assembly with the NR1 subunit is required for surface expression of NR3A-containing NMDA receptors. J. Neurosci. 21, 1228-1237 (2001).
    • (2001) J. Neurosci , vol.21 , pp. 1228-1237
    • Perez-Otaño, I.1
  • 23
    • 71949120013 scopus 로고    scopus 로고
    • Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with rab11 activity
    • Li, X. et al. Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with Rab11 activity. Mol. Cell Biol. 29, 6106-6116 (2009).
    • (2009) Mol. Cell Biol , vol.29 , pp. 6106-6116
    • Li, X.1
  • 25
    • 0037009161 scopus 로고    scopus 로고
    • Temporal and regional expression of nmda receptor subunit nr3a in the mammalian brain
    • Wong, H.K. et al. Temporal and regional expression of NMDA receptor subunit NR3A in the mammalian brain. J. Comp. Neurol. 450, 303-317 (2002).
    • (2002) J. Comp. Neurol , vol.450 , pp. 303-317
    • Wong, H.K.1
  • 27
    • 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. 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 87, 493-506 (1996).
    • (1996) Cell , vol.87 , pp. 493-506
    • Mangiarini, L.1
  • 28
    • 0032919866 scopus 로고    scopus 로고
    • Syndapin i a synaptic dynamin-binding protein that associates with the neural Wiskott-Aldrich syndrome protein
    • Qualmann, B., Roos, J., DiGregorio, P.J. & Kelly, R.B. Syndapin I, a synaptic dynamin-binding protein that associates with the neural Wiskott-Aldrich syndrome protein. Mol. Biol. Cell 10, 501-513 (1999).
    • (1999) Mol. Biol. Cell , vol.10 , pp. 501-513
    • Qualmann, B.1    Roos, J.2    Digregorio, P.J.3    Kelly, R.B.4
  • 29
    • 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
    • Canals, J.M. et al. Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington's disease. J. Neurosci. 24, 7727-7739 (2004).
    • (2004) J. Neurosci , vol.24 , pp. 7727-7739
    • Canals, J.M.1
  • 30
    • 16244392082 scopus 로고    scopus 로고
    • Deficits in experience-dependent cortical plasticity and sensory-discrimination learning in presymptomatic Huntington's disease mice
    • Mazarakis, N.K. et al. Deficits in experience-dependent cortical plasticity and sensory-discrimination learning in presymptomatic Huntington's disease mice. J. Neurosci. 25, 3059-3066 (2005).
    • (2005) J. Neurosci , vol.25 , pp. 3059-3066
    • Mazarakis, N.K.1
  • 31
    • 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. et al. Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain. Neurobiol. Dis. 26, 189-200 (2007).
    • (2007) Neurobiol. Dis , vol.26 , pp. 189-200
    • Van Raamsdonk, J.M.1
  • 32
    • 30744459353 scopus 로고    scopus 로고
    • Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models
    • Graham, R.K. et al. Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models. Neurobiol. Dis. 21, 444-455 (2006).
    • (2006) Neurobiol. Dis , vol.21 , pp. 444-455
    • Graham, R.K.1
  • 33
    • 0037087771 scopus 로고    scopus 로고
    • Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
    • Wheeler, V.C. et al. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum. Mol. Genet. 11, 633-640 (2002).
    • (2002) Hum. Mol. Genet , vol.11 , pp. 633-640
    • Wheeler, V.C.1
  • 34
    • 84864823046 scopus 로고    scopus 로고
    • The nmda receptor subunit glun3a protects against 3-nitroproprionic- induced striatal lesions via inhibition of calpain activation
    • Martínez-Turrillas, R. et al. The NMDA receptor subunit GluN3A protects against 3-nitroproprionic-induced striatal lesions via inhibition of calpain activation. Neurobiol. Dis. 48, 290-298 (2012).
    • (2012) Neurobiol. Dis , vol.48 , pp. 290-298
    • Martínez-Turrillas, R.1
  • 35
    • 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 227, 770-773 (1985).
    • (1985) Science , vol.227 , pp. 770-773
    • Graveland, G.A.1    Williams, R.S.2    Difiglia, M.3
  • 36
    • 77956240426 scopus 로고    scopus 로고
    • Alterations in striatal synaptic transmission are consistent across genetic mouse models of Huntington's disease
    • Cummings, D.M., Cepeda, C. & Levine, M.S. Alterations in striatal synaptic transmission are consistent across genetic mouse models of Huntington's disease. ASN Neuro 2, e00036 (2010).
    • (2010) ASN Neuro , vol.2
    • Cummings, D.M.1    Cepeda, C.2    Levine, M.S.3
  • 37
    • 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. et al. Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease. J. Neurosci. 25, 4169-4180 (2005).
    • (2005) J. Neurosci , vol.25 , pp. 4169-4180
    • Van Raamsdonk, J.M.1
  • 38
    • 0034612255 scopus 로고    scopus 로고
    • Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice
    • Bibb, J.A. et al. Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice. Proc. Natl. Acad. Sci. USA 97, 6809-6814 (2000).
    • (2000) Proc. Natl. Acad. Sci. USA , vol.97 , pp. 6809-6814
    • Bibb, J.A.1
  • 39
    • 36949012566 scopus 로고    scopus 로고
    • Huntington disease models and human neuropathology: Similarities and differences
    • Vonsattel, J.P. Huntington disease models and human neuropathology: similarities and differences. Acta Neuropathol. 115, 55-69 (2008).
    • (2008) Acta Neuropathol , vol.115 , pp. 55-69
    • Vonsattel, J.P.1
  • 41
    • 79955959358 scopus 로고    scopus 로고
    • Identification of anti-inflammatory targets for Huntington's disease using a brain slice-based screening assay
    • Reinhart, P.H. et al. Identification of anti-inflammatory targets for Huntington's disease using a brain slice-based screening assay. Neurobiol. Dis. 43, 248-256 (2011).
    • (2011) Neurobiol. Dis , vol.43 , pp. 248-256
    • Reinhart, P.H.1
  • 42
    • 23844472610 scopus 로고    scopus 로고
    • Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions
    • Slow, E.J. et al. Absence of behavioral abnormalities and neurodegeneration in vivo despite widespread neuronal huntingtin inclusions. Proc. Natl. Acad. Sci. USA 102, 11402-11407 (2005).
    • (2005) Proc. Natl. Acad. Sci. USA , vol.102 , pp. 11402-11407
    • Slow, E.J.1
  • 43
    • 71549143207 scopus 로고    scopus 로고
    • Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin
    • Okamoto, S. et al. Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin. Nat. Med. 15, 1407-1413 (2009).
    • (2009) Nat. Med , vol.15 , pp. 1407-1413
    • Okamoto, S.1
  • 44
    • 33645235438 scopus 로고    scopus 로고
    • Pharmacological promotion of inclusion formation: A therapeutic approach for huntington's and parkinson's diseases
    • Bodner, R.A. et al. Pharmacological promotion of inclusion formation: a therapeutic approach for Huntington's and Parkinson's diseases. Proc. Natl. Acad. Sci. USA 103, 4246-4251 (2006).
    • (2006) Proc. Natl. Acad. Sci. USA , vol.103 , pp. 4246-4251
    • Bodner, R.A.1
  • 45
    • 14844314896 scopus 로고    scopus 로고
    • Enhanced striatal nr2b-containing n-methyl-d-aspartate receptor-mediated synaptic currents in a mouse model of Huntington disease
    • Li, L., Murphy, T.H., Hayden, M.R. & Raymond, L.A. Enhanced striatal NR2B-containing N-methyl-d-aspartate receptor-mediated synaptic currents in a mouse model of Huntington disease. J. Neurophysiol. 92, 2738-2746 (2004).
    • (2004) J. Neurophysiol , vol.92 , pp. 2738-2746
    • Li, L.1    Murphy, T.H.2    Hayden, M.R.3    Raymond, L.A.4
  • 46
    • 0034234519 scopus 로고    scopus 로고
    • Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human huntington's disease mutation
    • Murphy, K.P. et al. Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation. J. Neurosci. 20, 5115-5123 (2000).
    • (2000) J. Neurosci , vol.20 , pp. 5115-5123
    • Murphy, K.P.1
  • 47
    • 0032897760 scopus 로고    scopus 로고
    • Impaired synaptic plasticity in mice carrying the Huntington's disease mutation
    • Usdin, M.T., Shelbourne, P.F., Myers, R.M. & Madison, D.V. Impaired synaptic plasticity in mice carrying the Huntington's disease mutation. Hum. Mol. Genet. 8, 839-846 (1999).
    • (1999) Hum. Mol. Genet , vol.8 , pp. 839-846
    • Usdin, M.T.1    Shelbourne, P.F.2    Myers, R.M.3    Madison, D.V.4
  • 48
    • 34247402598 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor restores synaptic plasticity in a knock-in mouse model of huntington's disease
    • Lynch, G. et al. Brain-derived neurotrophic factor restores synaptic plasticity in a knock-in mouse model of Huntington's disease. J. Neurosci. 27, 4424-4434 (2007).
    • (2007) J. Neurosci , vol.27 , pp. 4424-4434
    • Lynch, G.1
  • 49
    • 27644478238 scopus 로고    scopus 로고
    • Alterations in N-methyl-d-aspartate receptor sensitivity and magnesium blockade occur early in development in the R6/2 mouse model of Huntington's disease
    • Starling, A.J. et al. Alterations in N-methyl-d-aspartate receptor sensitivity and magnesium blockade occur early in development in the R6/2 mouse model of Huntington's disease. J. Neurosci. Res. 82, 377-386 (2005).
    • (2005) J. Neurosci. Res , vol.82 , pp. 377-386
    • Starling, A.J.1
  • 50
    • 77956917231 scopus 로고    scopus 로고
    • Synaptic versus extrasynaptic NMDA receptor signalling: Implications for neurodegenerative disorders
    • Hardingham, G.E. & Bading, H. Synaptic versus extrasynaptic NMDA receptor signalling: implications for neurodegenerative disorders. Nat. Rev. Neurosci. 11, 682-696 (2010).
    • (2010) Nat. Rev. Neurosci , vol.11 , pp. 682-696
    • Hardingham, G.E.1    Bading, H.2
  • 52
    • 65549107439 scopus 로고    scopus 로고
    • Neuroprotection by the nr3a subunit of the nmda receptor
    • Nakanishi, N. et al. Neuroprotection by the NR3A subunit of the NMDA receptor. J. Neurosci. 29, 5260-5265 (2009).
    • (2009) J. Neurosci , vol.29 , pp. 5260-5265
    • Nakanishi, N.1
  • 53
    • 0033587760 scopus 로고    scopus 로고
    • Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity
    • Hansson, O. et al. Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity. Proc. Natl. Acad. Sci. USA 96, 8727-8732 (1999).
    • (1999) Proc. Natl. Acad. Sci. USA , vol.96 , pp. 8727-8732
    • Hansson, O.1
  • 54
    • 60849093185 scopus 로고    scopus 로고
    • Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease
    • Graham, R.K. et al. Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease. J. Neurosci. 29, 2193-2204 (2009).
    • (2009) J. Neurosci , vol.29 , pp. 2193-2204
    • Graham, R.K.1
  • 55
    • 14044266141 scopus 로고    scopus 로고
    • Rna association and nucleocytoplasmic shuttling by ataxin-1
    • Irwin, S. et al. RNA association and nucleocytoplasmic shuttling by ataxin-1. J. Cell Sci. 118, 233-242 (2005).
    • (2005) J. Cell Sci , vol.118 , pp. 233-242
    • Irwin, S.1
  • 56
    • 33745593763 scopus 로고    scopus 로고
    • Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice
    • Lloret, A. et al. Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice. Hum. Mol. Genet. 15, 2015-2024 (2006).
    • (2006) Hum. Mol. Genet , vol.15 , pp. 2015-2024
    • Lloret, A.1
  • 57
    • 0037129830 scopus 로고    scopus 로고
    • Analysis of glutamate receptor surface expression in acute hippocampal slices
    • Grosshans, D.R., Clayton, D.A., Coultrap, S.J. & Browning, M.D. Analysis of glutamate receptor surface expression in acute hippocampal slices. Sci. STKE 2002, Pl8 (2002).
    • (2002) Sci. STKE , vol.2002
    • Grosshans, D.R.1    Clayton, D.A.2    Coultrap, S.J.3    Browning, M.D.4
  • 58
    • 80053613635 scopus 로고    scopus 로고
    • Conditional BDNF release under pathological conditions improves Huntington's disease pathology by delaying neuronal dysfunction
    • Giralt, A., Carreton, O., Lao-Peregrin, C., Martin, E.D. & Alberch, J. Conditional BDNF release under pathological conditions improves Huntington's disease pathology by delaying neuronal dysfunction. Mol. Neurodegener. 6, 71 (2011).
    • (2011) Mol. Neurodegener , vol.6 , pp. 71
    • Giralt, A.1    Carreton, O.2    Lao-Peregrin, C.3    Martin, E.D.4    Alberch, J.5
  • 59
    • 84859712559 scopus 로고    scopus 로고
    • Phenylbutyrate rescues dendritic spine loss associated with memory deficits in a mouse model of Alzheimer disease
    • Ricobaraza, A., Cuadrado-Tejedor, M., Marco, S., Perez-Otano, I. & Garcia-Osta, A. Phenylbutyrate rescues dendritic spine loss associated with memory deficits in a mouse model of Alzheimer disease. Hippocampus 22, 1040-1050 (2012).
    • (2012) Hippocampus , vol.22 , pp. 1040-1050
    • Ricobaraza, A.1    Cuadrado-Tejedor, M.2    Marco, S.3    Perez-Otano, I.4    Garcia-Osta, A.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.