메뉴 건너뛰기




Volumn 23, Issue 6, 2003, Pages 2193-2202

Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease

Author keywords

Apoptosis; Huntington; Neurodegeneration; Polyglutamine; Transgenic; Ultrastructure

Indexed keywords

CASPASE; GLIAL FIBRILLARY ACIDIC PROTEIN; HUNTINGTIN; MUTANT PROTEIN;

EID: 0037444445     PISSN: 02706474     EISSN: None     Source Type: Journal    
DOI: 10.1523/jneurosci.23-06-02193.2003     Document Type: Article
Times cited : (223)

References (55)
  • 2
    • 0034237719 scopus 로고    scopus 로고
    • Energetics in the pathogenesis of neurodegenerative diseases
    • Beal MF (2000) Energetics in the pathogenesis of neurodegenerative diseases. Trends Neurosci 23:298-304.
    • (2000) Trends Neurosci , vol.23 , pp. 298-304
    • Beal, M.F.1
  • 3
    • 0035947372 scopus 로고    scopus 로고
    • Impairment of the ubiquitin-proteasome system by protein aggregation
    • Bence NF, Sampat RM, Kopito RR (2001) Impairment of the ubiquitin-proteasome system by protein aggregation. Science 292:1552-1555.
    • (2001) Science , vol.292 , pp. 1552-1555
    • Bence, N.F.1    Sampat, R.M.2    Kopito, R.R.3
  • 7
    • 0025239753 scopus 로고
    • Degeneration of pyramidal projection neurons in Huntington's disease cortex
    • Cudkowicz M, Kowall NW (1990) Degeneration of pyramidal projection neurons in Huntington's disease cortex. Ann Neurol 27:200-204.
    • (1990) Ann Neurol , vol.27 , pp. 200-204
    • Cudkowicz, M.1    Kowall, N.W.2
  • 9
    • 0030752709 scopus 로고    scopus 로고
    • Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
    • DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, Aronin N (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 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
  • 10
    • 0029040355 scopus 로고
    • In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes
    • Dragunow M, Faull RL, Lawlor P, Beilharz EJ, Singleton K, Walker EB, Mee E (1995) In situ evidence for DNA fragmentation in Huntington's disease striatum and Alzheimer's disease temporal lobes. NeuroReport 6:1053-1057.
    • (1995) NeuroReport , vol.6 , pp. 1053-1057
    • Dragunow, M.1    Faull, R.L.2    Lawlor, P.3    Beilharz, E.J.4    Singleton, K.5    Walker, E.B.6    Mee, E.7
  • 12
    • 0037096376 scopus 로고    scopus 로고
    • Calpain activation in Huntington's disease
    • Gafni J, Ellerby LM (2002) Calpain activation in Huntington's disease. J Neurosci 22:4842-4849.
    • (2002) J Neurosci , vol.22 , pp. 4842-4849
    • Gafni, J.1    Ellerby, L.M.2
  • 15
    • 0032946228 scopus 로고    scopus 로고
    • In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease
    • Hackam AS, Singaraja R, Zhang T, Gan L, Hayden MR (1999) In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease. Hum Mol Genet 8:25-33.
    • (1999) Hum Mol Genet , vol.8 , pp. 25-33
    • Hackam, A.S.1    Singaraja, R.2    Zhang, T.3    Gan, L.4    Hayden, M.R.5
  • 17
    • 0028799793 scopus 로고
    • Early loss of neostriatal striosome neurons in Huntington's disease
    • Hedreen JC, Folstein SE (1995) Early loss of neostriatal striosome neurons in Huntington's disease. J Neuropathol Exp Neurol 54:105-120.
    • (1995) J Neuropathol Exp Neurol , vol.54 , pp. 105-120
    • Hedreen, J.C.1    Folstein, S.E.2
  • 24
    • 0036677435 scopus 로고    scopus 로고
    • Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease
    • Keene CD, Rodrigues CM, Eich T, Chhabra MS, Steer CJ, Low WC (2002) Tauroursodeoxycholic acid, a bile acid, is neuroprotective in a transgenic animal model of Huntington's disease. Proc Natl Acad Sci USA 99:10671-10676.
    • (2002) Proc Natl Acad Sci USA , vol.99 , pp. 10671-10676
    • Keene, C.D.1    Rodrigues, C.M.2    Eich, T.3    Chhabra, M.S.4    Steer, C.J.5    Low, W.C.6
  • 25
    • 0035940412 scopus 로고    scopus 로고
    • Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis
    • Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB, Qin ZH, Aronin N, DiFiglia M (2001) Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc Natl Acad Sci USA 98:12784-12789.
    • (2001) Proc Natl Acad Sci USA , vol.98 , pp. 12784-12789
    • Kim, Y.J.1    Yi, Y.2    Sapp, E.3    Wang, Y.4    Cuiffo, B.5    Kegel, K.B.6    Qin, Z.H.7    Aronin, N.8    DiFiglia, M.9
  • 29
    • 0034426013 scopus 로고    scopus 로고
    • Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity
    • Li H, Li SH, Johnston H, Shelbourne PF, Li XJ (2000) Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity. Nat Genet 25:385-389.
    • (2000) Nat Genet , vol.25 , pp. 385-389
    • Li, H.1    Li, S.H.2    Johnston, H.3    Shelbourne, P.F.4    Li, X.J.5
  • 30
    • 0033168302 scopus 로고    scopus 로고
    • Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin
    • Li SH, Cheng AL, Li H, Li XJ (1999) Cellular defects and altered gene expression in PC12 cells stably expressing mutant huntingtin. J Neurosci 19:5159-5172.
    • (1999) J Neurosci , vol.19 , pp. 5159-5172
    • Li, S.H.1    Cheng, A.L.2    Li, H.3    Li, X.J.4
  • 31
    • 0034703874 scopus 로고    scopus 로고
    • Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis
    • Li SH, Lam S, Cheng AL, Li XJ (2000) Intranuclear huntingtin increases the expression of caspase-1 and induces apoptosis. Hum Mol Genet 9:2859-2867.
    • (2000) Hum Mol Genet , vol.9 , pp. 2859-2867
    • Li, S.H.1    Lam, S.2    Cheng, A.L.3    Li, X.J.4
  • 34
  • 38
    • 0037107191 scopus 로고    scopus 로고
    • Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice
    • Menalled LB, Sison JD, Wu Y, Olivieri M, Li XJ, Li H, Zeitlin S, Chesselet MF (2002) Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice. J Neurosci 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
  • 39
    • 0035869544 scopus 로고    scopus 로고
    • Tissue-specific proteolysis of Huntingtin (htt) in human brain: Evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum
    • Mende-Mueller LM, Toneff T, Hwang SR, Chesselet MF, Hook VY (2001) Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington's disease striatum. J Neurosci 21:1830-1837.
    • (2001) J Neurosci , vol.21 , pp. 1830-1837
    • Mende-Mueller, L.M.1    Toneff, T.2    Hwang, S.R.3    Chesselet, M.F.4    Hook, V.Y.5
  • 40
    • 0036606540 scopus 로고    scopus 로고
    • ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats
    • Nishitoh H, Matsuzawa A, Tobiume K, Saegusa K, Takeda K, Inoue K, Hori S, Kakizuka A, Ichijo H (2002) ASK1 is essential for endoplasmic reticulum stress-induced neuronal cell death triggered by expanded polyglutamine repeats. Genes Dev 16:1345-1355.
    • (2002) Genes Dev , vol.16 , pp. 1345-1355
    • Nishitoh, H.1    Matsuzawa, A.2    Tobiume, K.3    Saegusa, K.4    Takeda, K.5    Inoue, K.6    Hori, S.7    Kakizuka, A.8    Ichijo, H.9
  • 43
    • 0035871295 scopus 로고    scopus 로고
    • Beyond the Qs in the polyglutamine diseases
    • Orr HT (2001) Beyond the Qs in the polyglutamine diseases. Genes Dev 15:925-932.
    • (2001) Genes Dev , vol.15 , pp. 925-932
    • Orr, H.T.1
  • 45
    • 0029072690 scopus 로고
    • Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models
    • Portera-Cailliau C, Hedreen JC, Price DL, Koliatsos VE (1995) Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models. J Neurosci 15:3775-3787.
    • (1995) J Neurosci , vol.15 , pp. 3775-3787
    • Portera-Cailliau, C.1    Hedreen, J.C.2    Price, D.L.3    Koliatsos, V.E.4
  • 47
    • 0033103523 scopus 로고    scopus 로고
    • Caspase-8 is required for cell death induced by expanded polyglutamine repeats
    • Sanchez I, Xu CJ, Juo P, Kakizaka A, Blenis J, Yuan J (1999) Caspase-8 is required for cell death induced by expanded polyglutamine repeats. Neuron 22:623-633.
    • (1999) Neuron , vol.22 , pp. 623-633
    • Sanchez, I.1    Xu, C.J.2    Juo, P.3    Kakizaka, A.4    Blenis, J.5    Yuan, J.6
  • 49
    • 0027377217 scopus 로고
    • Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington's disease: A quantitative Golgi study
    • Sotrel A, Williams RS, Kaufmann WE, Myers RH (1993) Evidence for neuronal degeneration and dendritic plasticity in cortical pyramidal neurons of Huntington's disease: a quantitative Golgi study. Neurology 43:2088-2096.
    • (1993) Neurology , vol.43 , pp. 2088-2096
    • Sotrel, A.1    Williams, R.S.2    Kaufmann, W.E.3    Myers, R.H.4
  • 55
    • 0035930598 scopus 로고    scopus 로고
    • Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation
    • Zhou H, Li SH, Li XJ (2001) Chaperone suppression of cellular toxicity of huntingtin is independent of polyglutamine aggregation. J Biol Chem 276:48417-48424.
    • (2001) J Biol Chem , vol.276 , pp. 48417-48424
    • Zhou, H.1    Li, S.H.2    Li, X.J.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.