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Volumn 7, Issue , 2006, Pages

Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease

Author keywords

[No Author keywords available]

Indexed keywords

HUNTINGTIN; MUTANT PROTEIN; PHOSPHOPROTEIN DARPP 32;

EID: 33846576161     PISSN: None     EISSN: 14712202     Source Type: Journal    
DOI: 10.1186/1471-2202-7-80     Document Type: Article
Times cited : (47)

References (42)
  • 1
    • 0033136692 scopus 로고    scopus 로고
    • A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
    • 10.1016/S0896-6273(00)80764-3 10402204
    • Hodgson JG Agopyan N Gutekunst CA Leavitt BR LePiane F Singaraja R A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration Neuron 1999 23 181-192 10.1016/S0896-6273(00)80764-3 10402204
    • (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
  • 2
    • 17344367977 scopus 로고    scopus 로고
    • Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA
    • 10.1038/2510 9771716
    • Reddy PH Williams M Charles V Garrett L Pike-Buchanan L Whetsell WO Jr Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA Nat Genet 1998 20 198-202 10.1038/2510 9771716
    • (1998) Nat Genet , vol.20 , pp. 198-202
    • Reddy, P.H.1    Williams, M.2    Charles, V.3    Garrett, L.4    Pike-Buchanan, L.5    Whetsell Jr., W.O.6
  • 3
    • 10744227174 scopus 로고    scopus 로고
    • Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
    • 10.1093/hmg/ddg169 12812983
    • Slow EJ van Raamsdonk J Rogers D Coleman SH Graham RK Deng Y Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease Hum Mol Genet 2003 12 1555-1567 10.1093/hmg/ddg169 12812983
    • (2003) Hum Mol Genet , vol.12 , pp. 1555-1567
    • Slow, E.J.1    van Raamsdonk, J.2    Rogers, D.3    Coleman, S.H.4    Graham, R.K.5    Deng, Y.6
  • 4
    • 0035282594 scopus 로고    scopus 로고
    • Loss of normal huntingtin function: New developments in Huntington's disease research
    • 10.1016/S0166-2236(00)01721-5 11182459
    • Cattaneo E Rigamonti D Goffredo D Zuccato C Squitieri F Sipione S Loss of normal huntingtin function: New developments in Huntington's disease research Trends Neurosci 2001 24 182-188 10.1016/S0166-2236(00)01721-5 11182459
    • (2001) Trends Neurosci , vol.24 , pp. 182-188
    • Cattaneo, E.1    Rigamonti, D.2    Goffredo, D.3    Zuccato, C.4    Squitieri, F.5    Sipione, S.6
  • 5
    • 19744380273 scopus 로고    scopus 로고
    • Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease
    • 10.1093/hmg/ddi147 15829505
    • Van Raamsdonk JM Pearson J Rogers DA Bissada N Vogl AW Hayden MR Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease Hum Mol Genet 2005 14 1379-1392 10.1093/hmg/ddi147 15829505
    • (2005) Hum Mol Genet , vol.14 , pp. 1379-1392
    • Van Raamsdonk, J.M.1    Pearson, J.2    Rogers, D.A.3    Bissada, N.4    Vogl, A.W.5    Hayden, M.R.6
  • 6
    • 0029082383 scopus 로고
    • Inactivation of the mouse Huntington's disease gene homolog Hdh
    • 10.1126/science.7618107 7618107
    • Duyao MP Auerbach AB Ryan A Persichetti F Barnes GT McNeil SM Inactivation of the mouse Huntington's disease gene homolog Hdh Science 1995 269 407-410 10.1126/science.7618107 7618107
    • (1995) Science , vol.269 , pp. 407-410
    • Duyao, M.P.1    Auerbach, A.B.2    Ryan, A.3    Persichetti, F.4    Barnes, G.T.5    McNeil, S.M.6
  • 7
    • 0029055717 scopus 로고
    • Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
    • 10.1016/0092-8674(95)90542-1 7774020
    • Nasir J Floresco SB O'Kusky JR Diewert VM Richman JM Zeisler J Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes Cell 1995 81 811-823 10.1016/0092-8674(95)90542-1 7774020
    • (1995) Cell , vol.81 , pp. 811-823
    • Nasir, J.1    Floresco, S.B.2    O'Kusky, J.R.3    Diewert, V.M.4    Richman, J.M.5    Zeisler, J.6
  • 8
    • 84993912315 scopus 로고
    • Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue
    • 10.1038/ng1095-155 7550343
    • Zeitlin S Liu JP Chapman DL Papaioannou VE Efstratiadis A Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue Nat Genet 1995 11 155-163 10.1038/ ng1095-155 7550343
    • (1995) Nat Genet , vol.11 , pp. 155-163
    • Zeitlin, S.1    Liu, J.P.2    Chapman, D.L.3    Papaioannou, V.E.4    Efstratiadis, A.5
  • 9
    • 0033550705 scopus 로고    scopus 로고
    • Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington's disease gene
    • 10.1016/S0006-8993(98)01312-2 10082833
    • O'Kusky JR Nasir J Cicchetti F Parent A Hayden MR Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington's disease gene Brain Res 1999 818 468-479 10.1016/S0006-8993(98)01312-2 10082833
    • (1999) Brain Res , vol.818 , pp. 468-479
    • O'Kusky, J.R.1    Nasir, J.2    Cicchetti, F.3    Parent, A.4    Hayden, M.R.5
  • 10
    • 0030613177 scopus 로고    scopus 로고
    • Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion
    • 10.1038/ng1297-404 9398841
    • White JK Auerbach W Duyao MP Vonsattel JP Gusella JF Joyner AL Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion Nat Genet 1997 17 404-410 10.1038/ ng1297-404 9398841
    • (1997) Nat Genet , vol.17 , pp. 404-410
    • White, J.K.1    Auerbach, W.2    Duyao, M.P.3    Vonsattel, J.P.4    Gusella, J.F.5    Joyner, A.L.6
  • 11
    • 0033757718 scopus 로고    scopus 로고
    • Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
    • 10.1038/81593 11062468
    • Dragatsis I Levine MS Zeitlin S Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice Nat Genet 2000 26 300-306 10.1038/81593 11062468
    • (2000) Nat Genet , vol.26 , pp. 300-306
    • Dragatsis, I.1    Levine, M.S.2    Zeitlin, S.3
  • 13
    • 0034933959 scopus 로고    scopus 로고
    • Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease
    • 10.1136/jmg.38.7.450 11432963
    • Ho LW Brown R Maxwell M Wyttenbach A Rubinsztein DC Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington's disease J Med Genet 2001 38 450-452 10.1136/ jmg.38.7.450 11432963
    • (2001) J Med Genet , vol.38 , pp. 450-452
    • Ho, L.W.1    Brown, R.2    Maxwell, M.3    Wyttenbach, A.4    Rubinsztein, D.C.5
  • 14
    • 0035127907 scopus 로고    scopus 로고
    • Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo
    • 1235265 11133364 10.1086/318207
    • Leavitt BR Guttman JA Hodgson JG Kimel GH Singaraja R Vogl AW Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo Am J Hum Genet 2001 68 313-324 1235265 11133364 10.1086/318207
    • (2001) Am J Hum Genet , vol.68 , pp. 313-324
    • Leavitt, B.R.1    Guttman, J.A.2    Hodgson, J.G.3    Kimel, G.H.4    Singaraja, R.5    Vogl, A.W.6
  • 15
    • 0034731370 scopus 로고    scopus 로고
    • Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain
    • 10.1074/jbc.M008408200 11007801
    • Hackam AS Yassa AS Singaraja R Metzler M Gutekunst CA Gan L Huntingtin interacting protein 1 induces apoptosis via a novel caspase-dependent death effector domain J Biol Chem 2000 275 41299-41308 10.1074/ jbc.M008408200 11007801
    • (2000) J Biol Chem , vol.275 , pp. 41299-41308
    • Hackam, A.S.1    Yassa, A.S.2    Singaraja, R.3    Metzler, M.4    Gutekunst, C.A.5    Gan, L.6
  • 16
    • 3142636768 scopus 로고    scopus 로고
    • Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
    • 10.1016/j.cell.2004.06.018 15242649
    • Gauthier LR Charrin BC Borrell-Pages M Dompierre JP Rangone H Cordelieres FP Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules Cell 2004 118 127-138 10.1016/j.cell.2004.06.018 15242649
    • (2004) Cell , vol.118 , pp. 127-138
    • Gauthier, L.R.1    Charrin, B.C.2    Borrell-Pages, M.3    Dompierre, J.P.4    Rangone, H.5    Cordelieres, F.P.6
  • 17
    • 0035919701 scopus 로고    scopus 로고
    • Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease
    • 10.1126/science.1059581 11408619
    • Zuccato C Ciammola A Rigamonti D Leavitt BR Goffredo D Conti L Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease Science 2001 293 493-498 10.1126/science.1059581 11408619
    • (2001) Science , vol.293 , pp. 493-498
    • Zuccato, C.1    Ciammola, A.2    Rigamonti, D.3    Leavitt, B.R.4    Goffredo, D.5    Conti, L.6
  • 18
    • 0041353535 scopus 로고    scopus 로고
    • Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes
    • 10.1038/ng1219 12881722
    • Zuccato C Tartari M Crotti A Goffredo D Valenza M Conti L Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes Nat Genet 2003 35 76-83 10.1038/ng1219 12881722
    • (2003) Nat Genet , vol.35 , pp. 76-83
    • Zuccato, C.1    Tartari, M.2    Crotti, A.3    Goffredo, D.4    Valenza, M.5    Conti, L.6
  • 19
    • 0033587760 scopus 로고    scopus 로고
    • Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity
    • 17584 10411943 10.1073/pnas.96.15.8727
    • Hansson O Petersen A Leist M Nicotera P Castilho RF Brundin P Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity Proc Natl Acad Sci USA 1999 96 8727-8732 17584 10411943 10.1073/pnas.96.15.8727
    • (1999) Proc Natl Acad Sci USA , vol.96 , pp. 8727-8732
    • Hansson, O.1    Petersen, A.2    Leist, M.3    Nicotera, P.4    Castilho, R.F.5    Brundin, P.6
  • 20
    • 4444302167 scopus 로고    scopus 로고
    • Deranged neuronal calcium signaling and Huntington disease
    • 10.1016/j.bbrc.2004.08.035 15336977
    • Bezprozvanny I Hayden MR Deranged neuronal calcium signaling and Huntington disease Biochem Biophys Res Commun 2004 322 1310-1317 10.1016/ j.bbrc.2004.08.035 15336977
    • (2004) Biochem Biophys Res Commun , vol.322 , pp. 1310-1317
    • Bezprozvanny, I.1    Hayden, M.R.2
  • 21
    • 0032900574 scopus 로고    scopus 로고
    • Biochemical abnormalities and excitotoxicity in Huntington's disease brain
    • 10.1002/1531-8249(199901)45:1<25::AID-ART6>3.0.CO;2-E 9894873
    • Tabrizi SJ Cleeter MW Xuereb J Taanman JW Cooper JM Schapira AH Biochemical abnormalities and excitotoxicity in Huntington's disease brain Ann Neurol 1999 45 25-32 10.1002/ 1531-8249(199901)45:1<25::AID-ART6>3.0.CO;2-E 9894873
    • (1999) Ann Neurol , vol.45 , pp. 25-32
    • Tabrizi, S.J.1    Cleeter, M.W.2    Xuereb, J.3    Taanman, J.W.4    Cooper, J.M.5    Schapira, A.H.6
  • 22
    • 10544222019 scopus 로고    scopus 로고
    • Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype
    • 10.1093/hmg/5.12.1875 8968738
    • Hodgson JG Smith DJ McCutcheon K Koide HB Nishiyama K Dinulos MB Human huntingtin derived from YAC transgenes compensates for loss of murine huntingtin by rescue of the embryonic lethal phenotype Hum Mol Genet 1996 5 1875-1885 10.1093/hmg/5.12.1875 8968738
    • (1996) Hum Mol Genet , vol.5 , pp. 1875-1885
    • Hodgson, J.G.1    Smith, D.J.2    McCutcheon, K.3    Koide, H.B.4    Nishiyama, K.5    Dinulos, M.B.6
  • 24
    • 1242338856 scopus 로고    scopus 로고
    • Huntingtin-protein interactions and the pathogenesis of Huntington's disease
    • 10.1016/j.tig.2004.01.008 15036808
    • Li SH Li XJ Huntingtin-protein interactions and the pathogenesis of Huntington's disease Trends Genet 2004 20 146-154 10.1016/ j.tig.2004.01.008 15036808
    • (2004) Trends Genet , vol.20 , pp. 146-154
    • Li, S.H.1    Li, X.J.2
  • 25
    • 17644368893 scopus 로고    scopus 로고
    • Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease
    • 10.1523/JNEUROSCI.0590-05.2005 15843620
    • Van Raamsdonk JM Pearson J Slow EJ Hossain SM Leavitt BR Hayden MR Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington's disease J Neurosci 2005 25 4169-4180 10.1523/JNEUROSCI.0590-05.2005 15843620
    • (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
  • 27
    • 9444239187 scopus 로고    scopus 로고
    • Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme
    • 10.1074/jbc.271.32.19385 8702625
    • Kalchman MA Graham RK Xia G Koide HB Hodgson JG Graham KC Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme J Biol Chem 1996 271 19385-19394 10.1074/jbc.271.32.19385 8702625
    • (1996) J Biol Chem , vol.271 , pp. 19385-19394
    • Kalchman, M.A.1    Graham, R.K.2    Xia, G.3    Koide, H.B.4    Hodgson, J.G.5    Graham, K.C.6
  • 28
    • 27744478499 scopus 로고    scopus 로고
    • Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease
    • 10.1016/j.expneurol.2005.07.021 16129433
    • Van Raamsdonk JM Pearson J Rogers DA Lu G Barakauskas VE Barr AM Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease Exp Neurol 2005 196 266-272 10.1016/j.expneurol.2005.07.021 16129433
    • (2005) Exp Neurol , vol.196 , pp. 266-272
    • Van Raamsdonk, J.M.1    Pearson, J.2    Rogers, D.A.3    Lu, G.4    Barakauskas, V.E.5    Barr, A.M.6
  • 29
    • 0142157600 scopus 로고    scopus 로고
    • Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice
    • 14561870
    • Ferrante RJ Kubilus JK Lee J Ryu H Beesen A Zucker B Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice J Neurosci 2003 23 9418-9427 14561870
    • (2003) J Neurosci , vol.23 , pp. 9418-9427
    • Ferrante, R.J.1    Kubilus, J.K.2    Lee, J.3    Ryu, H.4    Beesen, A.5    Zucker, B.6
  • 30
    • 20844455450 scopus 로고    scopus 로고
    • Chemotherapy for the brain: The antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease
    • 10.1523/JNEUROSCI.2599-04.2004 15548647
    • Ferrante RJ Ryu H Kubilus JK D'Mello S Sugars KL Lee J Chemotherapy for the brain: The antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease J Neurosci 2004 24 10335-10342 10.1523/JNEUROSCI.2599-04.2004 15548647
    • (2004) J Neurosci , vol.24 , pp. 10335-10342
    • Ferrante, R.J.1    Ryu, H.2    Kubilus, J.K.3    D'Mello, S.4    Sugars, K.L.5    Lee, J.6
  • 31
    • 19944431703 scopus 로고    scopus 로고
    • Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease
    • 15494404
    • Gardian G Browne SE Choi DK Klivenyi P Gregorio J Kubilus JK Neuroprotective effects of phenylbutyrate in the N171-82Q transgenic mouse model of Huntington's disease J Biol Chem 2005 280 556-563 15494404
    • (2005) J Biol Chem , vol.280 , pp. 556-563
    • Gardian, G.1    Browne, S.E.2    Choi, D.K.3    Klivenyi, P.4    Gregorio, J.5    Kubilus, J.K.6
  • 33
    • 0028316870 scopus 로고
    • A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats
    • 10.1056/NEJM199405193302001 8159192
    • Kremer B Goldberg P Andrew SE Theilmann J Telenius H Zeisler J 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 10.1056/NEJM199405193302001 8159192
    • (1994) N Engl J Med , vol.330 , pp. 1401-1406
    • Kremer, B.1    Goldberg, P.2    Andrew, S.E.3    Theilmann, J.4    Telenius, H.5    Zeisler, J.6
  • 34
    • 0033546889 scopus 로고    scopus 로고
    • DNA analysis of Huntington's disease: Five years of experience in Germany, Austria, and Switzerland
    • 10489044
    • Laccone F Engel U Holinski-Feder E Weigell-Weber M Marczinek K Nolte D DNA analysis of Huntington's disease: Five years of experience in Germany, Austria, and Switzerland Neurology 1999 53 801-806 10489044
    • (1999) Neurology , vol.53 , pp. 801-806
    • Laccone, F.1    Engel, U.2    Holinski-Feder, E.3    Weigell-Weber, M.4    Marczinek, K.5    Nolte, D.6
  • 36
    • 0037379416 scopus 로고    scopus 로고
    • Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course
    • 10.1093/brain/awg077 12615650
    • Squitieri F Gellera C Cannella M Mariotti C Cislaghi G Rubinsztein DC Homozygosity for CAG mutation in Huntington disease is associated with a more severe clinical course Brain 2003 126 946-955 10.1093/brain/awg077 12615650
    • (2003) Brain , vol.126 , pp. 946-955
    • Squitieri, F.1    Gellera, C.2    Cannella, M.3    Mariotti, C.4    Cislaghi, G.5    Rubinsztein, D.C.6
  • 39
    • 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
    • 10.1093/hmg/9.4.503 10699173
    • Wheeler VC White JK Gutekunst CA Vrbanac V Weaver M Li XJ 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 10.1093/hmg/9.4.503 10699173
    • (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
  • 40
    • 30744459353 scopus 로고    scopus 로고
    • Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models
    • 10.1016/j.nbd.2005.08.007 16230019
    • Graham RK Slow EJ Deng Y Bissada N Lu G Pearson J Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models Neurobiol Dis 2006 21 444-455 10.1016/ j.nbd.2005.08.007 16230019
    • (2006) Neurobiol Dis , vol.21 , pp. 444-455
    • Graham, R.K.1    Slow, E.J.2    Deng, Y.3    Bissada, N.4    Lu, G.5    Pearson, J.6
  • 41
    • 0035888618 scopus 로고    scopus 로고
    • The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin
    • 10.1093/hmg/10.22.2515 11709539
    • Auerbach W Hurlbert MS Hilditch-Maguire P Wadghiri YZ Wheeler VC Cohen SI The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin Hum Mol Genet 2001 10 2515-2523 10.1093/hmg/10.22.2515 11709539
    • (2001) Hum Mol Genet , vol.10 , pp. 2515-2523
    • Auerbach, W.1    Hurlbert, M.S.2    Hilditch-Maguire, P.3    Wadghiri, Y.Z.4    Wheeler, V.C.5    Cohen, S.I.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.