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Volumn 354, Issue 1386, 1999, Pages 1005-1011

Polyglutamine pathogenesis

Author keywords

Dentatorubral pallidoluysian atrophy; Huntington's disease; Neuronal toxicity; Transgenic mouse models

Indexed keywords

ANIMALIA; MUS MUSCULUS;

EID: 0033614776     PISSN: 09628436     EISSN: None     Source Type: Journal    
DOI: 10.1098/rstb.1999.0452     Document Type: Article
Times cited : (81)

References (82)
  • 1
    • 0027176364 scopus 로고
    • The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease
    • Andrew, S. E. (and 11 others) 1993 The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease. Nature Genet. 4, 398-403.
    • (1993) Nature Genet. , vol.4 , pp. 398-403
    • Andrew, S.E.1
  • 2
    • 0030700891 scopus 로고    scopus 로고
    • Rethinking genotype and phenotype correlations in polyglutamine expansion disorders
    • Andrew, S. E., Goldbery, Y., P. & Hayden, M. R. 1997 Rethinking genotype and phenotype correlations in polyglutamine expansion disorders. Hum. Mol. Genet. 6, 2005-2010.
    • (1997) Hum. Mol. Genet. , vol.6 , pp. 2005-2010
    • Andrew, S.E.1    Goldbery, Y.P.2    Hayden, M.R.3
  • 3
    • 0028829596 scopus 로고
    • CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain
    • Aronin, N. (and 16 others) 1995 CAG expansion affects the expression of mutant huntingtin in the Huntington's disease brain. Neuron 15, 1193-1201.
    • (1995) Neuron , vol.15 , pp. 1193-1201
    • Aronin, N.1
  • 4
    • 0031918640 scopus 로고    scopus 로고
    • Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: Correlation between the density of inclusions and IT15 triplet repeat length
    • Becher, M.W., Kotzuk, J. A., Sharp, A. H., Davies, S.W., Bates, G. P., Price, D. L. & Ross, C. A. 1998 Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 triplet repeat length, Neurobiol. Dis. 4, 387-397.
    • (1998) Neurobiol. Dis. , vol.4 , pp. 387-397
    • Becher, M.W.1    Kotzuk, J.A.2    Sharp, A.H.3    Davies, S.W.4    Bates, G.P.5    Price, D.L.6    Ross, C.A.7
  • 6
    • 0029129227 scopus 로고
    • Spinal and bulbar muscular atrophy: A trinucleotide-repeat expansion nuerodegenerative disease
    • Brooks, B. P. & Fischbeck, K. H. 1995 Spinal and bulbar muscular atrophy: a trinucleotide-repeat expansion nuerodegenerative disease. Trends Neurosci, 18, 459-461.
    • (1995) Trends Neurosci , vol.18 , pp. 459-461
    • Brooks, B.P.1    Fischbeck, K.H.2
  • 12
    • 0030767266 scopus 로고    scopus 로고
    • Huntingtin-associated protein 1 (HAP1) binds to a tri-like polypeptide, with a rac1 guanine nucleotide exchange factor domain
    • Colomer, V., Engelender, S., Sharp, A. H., Duan, K., Cooper, J. K., Lanahan, A., Lyford, G., Worley, P. & Ross, C. A. 1997 Huntingtin-associated protein 1 (HAP1) binds to a tri-like polypeptide, with a rac1 guanine nucleotide exchange factor domain. Hum. Mol. Genet. 6, 1519-1525.
    • (1997) Hum. Mol. Genet. , vol.6 , pp. 1519-1525
    • Colomer, V.1    Engelender, S.2    Sharp, A.H.3    Duan, K.4    Cooper, J.K.5    Lanahan, A.6    Lyford, G.7    Worley, P.8    Ross, C.A.9
  • 13
    • 7144253143 scopus 로고    scopus 로고
    • Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture
    • Cooper, J. K. (and 12 others) 1998 Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum. Mol. Genet. 7, 783-790.
    • (1998) Hum. Mol. Genet. , vol.7 , pp. 783-790
    • Cooper, J.K.1
  • 15
    • 0029816724 scopus 로고    scopus 로고
    • Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation
    • De Rooij, K. E., Dorsman, J. C., Smoor, M. A., Den Dunnen, J. T. & Van Ommen, G. J. 1996 Subcellular localization of the Huntington's disease gene product in cell lines by immunofluorescence and biochemical subcellular fractionation. Hum. Mol. Genet. 5, 1093-1099.
    • (1996) Hum. Mol. Genet. , vol.5 , pp. 1093-1099
    • De Rooij, K.E.1    Dorsman, J.C.2    Smoor, M.A.3    Den Dunnen, J.T.4    Van Ommen, G.J.5
  • 16
    • 0028989602 scopus 로고
    • Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
    • DiFiglia, M. (and 11 others) 1995 Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons. Neuron 14, 1075-1081.
    • (1995) Neuron , vol.14 , pp. 1075-1081
    • DiFiglia, M.1
  • 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., Rates, G. P., Vonsattel, J. P. & 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    Rates, G.P.5    Vonsattel, J.P.6    Aronin, N.7
  • 18
    • 0027240431 scopus 로고
    • Trinucleotide repeat length instability and age of onset in Huntington's disease
    • Duyao, M. (and 41 others) 1993 Trinucleotide repeat length instability and age of onset in Huntington's disease. Nature Genet. 4, 387-392.
    • (1993) Nature Genet. , vol.4 , pp. 387-392
    • Duyao, M.1
  • 21
    • 0033524413 scopus 로고    scopus 로고
    • Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc
    • Faber, P. W., Alter, J. R., MacDonald, M. E. & Hart, C. 1999 Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron. Proc. Natl Acad. Sci. USA 96, 179-184.
    • (1999) Natl Acad. Sci. USA , vol.96 , pp. 179-184
    • Faber, P.W.1    Alter, J.R.2    MacDonald, M.E.3    Hart, C.4
  • 23
    • 9344227302 scopus 로고    scopus 로고
    • Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract
    • Goldberg, Y. P. (and 10 others) 1996 Cleavage of huntingtin by apopain, a proapoptotic cysteine protease, is modulated by the polyglutamine tract. Nature Genet. 13, 442-449.
    • (1996) Nature Genet. , vol.13 , pp. 442-449
    • Goldberg, Y.P.1
  • 24
    • 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. 1985 Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease. Science 227, 770-773.
    • (1985) Science , vol.227 , pp. 770-773
    • Graveland, G.A.1    Williams, R.S.2    DiFiglia, M.3
  • 25
    • 0030948716 scopus 로고    scopus 로고
    • The genetic defect causing Huntington's disease: Repeated in other contexts?
    • Gusella, J. F., Persichetti, F. & MacDonald, M. E. 1997 The genetic defect causing Huntington's disease: repeated in other contexts? Mol. Med. 3, 238-246.
    • (1997) Mol. Med. , vol.3 , pp. 238-246
    • Gusella, J.F.1    Persichetti, F.2    MacDonald, M.E.3
  • 28
    • 0032946228 scopus 로고    scopus 로고
    • In vitro evidence for both the nucleus and cytoplasmas subcellular sites of pathogencsis in Huntington's disease
    • Hackam, A. S., Singaraja, R., Zhang, T., Gan, L. & Hayden, M. R. 1999 In vitro evidence for both the nucleus and cytoplasmas subcellular sites of pathogencsis 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
  • 29
    • 7144229376 scopus 로고    scopus 로고
    • Spinocerebellar ataxia type 7 (SCA7): A neurodegenerative disorder with neuronal intranuclear inclusions
    • Holmberg, M. (and 10 others) 1998 Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions. Hum. Mol. Genet. 7, 913-918.
    • (1998) Hum. Mol. Genet. , vol.7 , pp. 913-918
    • Holmberg, M.1
  • 31
    • 17344362229 scopus 로고    scopus 로고
    • Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch
    • Igarashi, S. (and 18 others) 1998 Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nature Genet. 18, 111-117.
    • (1998) Nature Genet. , vol.18 , pp. 111-117
    • Igarashi, S.1
  • 32
    • 0030058208 scopus 로고    scopus 로고
    • Expanded polyglutamine in the Machado Joseph disease protein induces cell death in vitro and in vivo
    • Ikeda, H., Yamaguchi, M., Sugai, S., Aze, Y., Narumiya, S. & Kakizuka, A. 1996 Expanded polyglutamine in the Machado Joseph disease protein induces cell death in vitro and in vivo. Nature Genet. 13, 196-202.
    • (1996) Nature Genet. , vol.13 , pp. 196-202
    • Ikeda, H.1    Yamaguchi, M.2    Sugai, S.3    Aze, Y.4    Narumiya, S.5    Kakizuka, A.6
  • 33
    • 0032168160 scopus 로고    scopus 로고
    • Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons
    • Jackson, G. R., Salecker, I., Dong, X., Yao, X., Arnheim, N., Faber, P. W., MacDonald, M. E. & Zipursky, S. L. 1998 Polyglutamine-expanded human huntingtin transgenes induce degeneration of Drosophila photoreceptor neurons. Neuron 21, 633-642.
    • (1998) Neuron , vol.21 , pp. 633-642
    • Jackson, G.R.1    Salecker, I.2    Dong, X.3    Yao, X.4    Arnheim, N.5    Faber, P.W.6    MacDonald, M.E.7    Zipursky, S.L.8
  • 35
    • 85020791481 scopus 로고    scopus 로고
    • HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membane-associated huntingtin in the brain
    • Kalchman, M. A. (and 13 others) 1997 HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membane-associated huntingtin in the brain. Nature Genet. 16, 4453.
    • (1997) Nature Genet. , vol.16 , pp. 4453
    • Kalchman, M.A.1
  • 36
    • 0032475941 scopus 로고    scopus 로고
    • Ataxin-1 nuclear localization and aggregation: Role in polyglutamine-induced disease in SCA1 transgenic mice
    • Klement, I. A., Skinner, P. J., Kaytor, M. D., Yi, H., Hersch, S. M., Clark, H. B., Zoghbi, H. Y. & Orr, H. T. 1998 Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95, 41-53.
    • (1998) Cell , vol.95 , pp. 41-53
    • Klement, I.A.1    Skinner, P.J.2    Kaytor, M.D.3    Yi, H.4    Hersch, S.M.5    Clark, H.B.6    Zoghbi, H.Y.7    Orr, H.T.8
  • 37
    • 0029833062 scopus 로고    scopus 로고
    • Spino-cerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase
    • Koshy, B., Matilla, T., Burright, E. N., Merry, D. E., Fischbeck, K. H., Orr, H. D. & Zoghbi, H. Y. 1996 Spino-cerebellar ataxia type-1 and spinobulbar muscular atrophy gene products interact with glyceraldehyde-3-phosphate dehydrogenase. Hum. Mol. Genet. 5, 1311-1318.
    • (1996) Hum. Mol. Genet. , vol.5 , pp. 1311-1318
    • Koshy, B.1    Matilla, T.2    Burright, E.N.3    Merry, D.E.4    Fischbeck, K.H.5    Orr, H.D.6    Zoghbi, H.Y.7
  • 38
    • 0344431341 scopus 로고    scopus 로고
    • Structural neurology: Are seeds at the root of neuronal degenertion?
    • Lansbury Jr, P. T. 1997 Structural neurology: are seeds at the root of neuronal degenertion? Neuron 19, 1151-1154.
    • (1997) Neuron , vol.19 , pp. 1151-1154
    • Lansbury P.T., Jr.1
  • 40
    • 0031945025 scopus 로고    scopus 로고
    • Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats
    • Li, S.-H. & Li, X.-J. 1998 Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats. Hum. Mol. Genet. 7, 777-782.
    • (1998) Hum. Mol. Genet. , vol.7 , pp. 777-782
    • Li, S.-H.1    Li, X.-J.2
  • 44
    • 0001388128 scopus 로고    scopus 로고
    • Expression of polyglutamine-expanded huntingtin activates the SEK1 JNK pathway and induces apoptosis in a hippocampal neuronal cell line
    • Liu, Y. F. 1998 Expression of polyglutamine-expanded huntingtin activates the SEK1 JNK pathway and induces apoptosis in a hippocampal neuronal cell line. J. Biol. Chem. 273, 28873-28877.
    • (1998) J. Biol. Chem. , vol.273 , pp. 28873-28877
    • Liu, Y.F.1
  • 45
    • 0000790724 scopus 로고    scopus 로고
    • SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes
    • Liu, Y. F., Deth, R. C. & Devys, D. 1997 SH3 domain-dependent association of huntingtin with epidermal growth factor receptor signaling complexes. J. Biol. Chem. 272, 8121-8124.
    • (1997) J. Biol. Chem. , vol.272 , pp. 8121-8124
    • Liu, Y.F.1    Deth, R.C.2    Devys, D.3
  • 46
    • 0031680014 scopus 로고    scopus 로고
    • A cellular model that recapitulates major pathogenic steps of Huntington's disease
    • Lunkes, A. & Mandel, J.-L. 1998 A cellular model that recapitulates major pathogenic steps of Huntington's disease. Hum. Mol. Genet. 7, 1355-1361.
    • (1998) Hum. Mol. Genet. , vol.7 , pp. 1355-1361
    • Lunkes, A.1    Mandel, J.-L.2
  • 47
    • 16044373842 scopus 로고    scopus 로고
    • Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phonotype in transgenic mice
    • Mangiarini, L. (and 10 others) 1996 Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phonotype in transgenic mice. Cell 87, 493-506.
    • (1996) Cell , vol.87 , pp. 493-506
    • Mangiarini, L.1
  • 48
    • 17344363559 scopus 로고    scopus 로고
    • Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates
    • Martindale, D. (and 12 others) 1998 Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nature Genet. 18, 150-154.
    • (1998) Nature Genet. , vol.18 , pp. 150-154
    • Martindale, D.1
  • 49
    • 0030716768 scopus 로고    scopus 로고
    • The cellular leucine rich acidic nuclear protein (LANP) interacts with ataxin-1: Further insight into the pathogenesis of SCA1
    • Matilla, A., Koshy, B., Cummings, C. J., Isobe, T., Orr, H. T. & Zoghbi, H. Y. 1997 The cellular leucine rich acidic nuclear protein (LANP) interacts with ataxin-1: further insight into the pathogenesis of SCA1. Nature 389, 974-978.
    • (1997) Nature , vol.389 , pp. 974-978
    • Matilla, A.1    Koshy, B.2    Cummings, C.J.3    Isobe, T.4    Orr, H.T.5    Zoghbi, H.Y.6
  • 50
    • 0031948607 scopus 로고
    • Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy
    • Merry, D. E., Kobayashi, Y., Bailey, C. K., Taye, A. A. & Fischbeck, K. H. 1990 Cleavage, aggregation and toxicity of the expanded androgen receptor in spinal and bulbar muscular atrophy. Hum. Mol. Genet. 7, 693-701.
    • (1990) Hum. Mol. Genet. , vol.7 , pp. 693-701
    • Merry, D.E.1    Kobayashi, Y.2    Bailey, C.K.3    Taye, A.A.4    Fischbeck, K.H.5
  • 51
    • 0030670816 scopus 로고    scopus 로고
    • Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis
    • Miyashita, T., Okamura-Oho, Y., Mito, Y., Nagafuchi, S. & Yamada, M. 1997 Dentatorubral pallidoluysian atrophy (DRPLA) protein is cleaved by caspase-3 during apoptosis. J. Biol. Chem. 272, 29238-29242.
    • (1997) J. Biol. Chem. , vol.272 , pp. 29238-29242
    • Miyashita, T.1    Okamura-Oho, Y.2    Mito, Y.3    Nagafuchi, S.4    Yamada, M.5
  • 52
    • 0032504549 scopus 로고    scopus 로고
    • Intracellular aggregate formation of dentatorubral pallidoluysian atrophy (DRPLA) protein with the extended polyglutamine
    • Miyashita, T., Nagao, K., Ohmi, K., Yanagisawa, H., Okamura-Oho, Y. & Yamada, M. 1998 Intracellular aggregate formation of dentatorubral pallidoluysian atrophy (DRPLA) protein with the extended polyglutamine. Biochem. Biophys. Res. Cumm. 249, 96-102.
    • (1998) Biochem. Biophys. Res. Cumm. , vol.249 , pp. 96-102
    • Miyashita, T.1    Nagao, K.2    Ohmi, K.3    Yanagisawa, H.4    Okamura-Oho, Y.5    Yamada, M.6
  • 53
    • 0033556160 scopus 로고    scopus 로고
    • Generation of neuronal intranuclear inclusions by polyglutamine-GFP: Analysis of inclusion clearance and toxicity as a function of polyglutamine length
    • Moulder, K. L., Onodera, O., Burke, J. R., Strittmatter, W. J. & Johnson Jr, E. M. 1999 Generation of neuronal intranuclear inclusions by polyglutamine-GFP: analysis of inclusion clearance and toxicity as a function of polyglutamine length. J. Neurosci. 19, 705-715.
    • (1999) J. Neurosci. , vol.19 , pp. 705-715
    • Moulder, K.L.1    Onodera, O.2    Burke, J.R.3    Strittmatter, W.J.4    Johnson E.M., Jr.5
  • 55
    • 0030848204 scopus 로고    scopus 로고
    • Clinical aspects of CAG repeat diseases
    • Nance, M. A. 1997 Clinical aspects of CAG repeat diseases. Brain Pathol, 7, 881-900.
    • (1997) Brain Pathol , vol.7 , pp. 881-900
    • Nance, M.A.1
  • 56
    • 0031469707 scopus 로고    scopus 로고
    • Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse
    • Ordway, J. M. (and 11 others) 1997 Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse. Cell 91, 753-763.
    • (1997) Cell , vol.91 , pp. 753-763
    • Ordway, J.M.1
  • 57
    • 0029968460 scopus 로고    scopus 로고
    • Trinucleotide repeats in neurogenetic disorders
    • Paulson, H. L. & Fischbeck, K. H. 1996 Trinucleotide repeats in neurogenetic disorders. A. Rev. Neurosci. 19, 79-107.
    • (1996) A. Rev. Neurosci. , vol.19 , pp. 79-107
    • Paulson, H.L.1    Fischbeck, K.H.2
  • 59
    • 0027988041 scopus 로고
    • Polar zippers: Their role in human disease
    • Perutz, M. 1991 Polar zippers: their role in human disease. Protein Sci. 3, 1629-1637.
    • (1991) Protein Sci. , vol.3 , pp. 1629-1637
    • Perutz, M.1
  • 62
    • 0029089172 scopus 로고
    • When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases
    • Ross, C., A. 1995 When more is less: pathogenesis of glutamine repeat neurodegenerative diseases. Neuron 15, 493-496.
    • (1995) Neuron , vol.15 , pp. 493-496
    • Ross, C.A.1
  • 63
    • 0031446233 scopus 로고    scopus 로고
    • Intranuclear neuronal inclusions: A common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?
    • Ross, C. A. 1997 Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases? Neuron 19, 1147-1150.
    • (1997) Neuron , vol.19 , pp. 1147-1150
    • Ross, C.A.1
  • 64
    • 0030868770 scopus 로고    scopus 로고
    • Reviews in molecular medicine: Huntington's disease and a related disorder, dentatorubral-pallidoluysian atrophy (DRPLA)
    • Ross, C. A., Margolis, R. L., Rosenblatt, A., Ranen, N. G., Becher, M. W. & Aylward, E. A. 1997 Reviews in molecular medicine: Huntington's disease and a related disorder, dentatorubral-pallidoluysian atrophy (DRPLA). Medicine 76, 305-338.
    • (1997) Medicine , vol.76 , pp. 305-338
    • Ross, C.A.1    Margolis, R.L.2    Rosenblatt, A.3    Ranen, N.G.4    Becher, M.W.5    Aylward, E.A.6
  • 65
    • 0009680381 scopus 로고    scopus 로고
    • Pathogenesis in triplet expansion disorders: Mechanism of polyglutamine-induced apoptosis
    • Sanchez, I. & Yuan, J. 1998 Pathogenesis in triplet expansion disorders: mechanism of polyglutamine-induced apoptosis. Soc. Neurosci. Abstr. 379.10.
    • (1998) Soc. Neurosci. Abstr. , vol.379 , pp. 10
    • Sanchez, I.1    Yuan, J.2
  • 67
    • 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. 1998 Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95, 55-66.
    • (1998) Cell , vol.95 , pp. 55-66
    • Saudou, F.1    Finkbeiner, S.2    Devys, D.3    Greenberg, M.E.4
  • 69
    • 0033054555 scopus 로고    scopus 로고
    • Intranuclear inclusions and neuritic pathology in transgenic mice expressing a mutant N-terminal fragment of huntingtin
    • In the press
    • Schilling, G. (and 13 others) 1999a Intranuclear inclusions and neuritic pathology in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum. Mol. Genet. (In the press.)
    • (1999) Hum. Mol. Genet.
    • Schilling, G.1
  • 70
    • 85020817124 scopus 로고    scopus 로고
    • Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA
    • Submitted
    • Schilling, G. (and 14 others) 1999b Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. Nature Genet. (Submitted.)
    • (1999) Nature Genet.
    • Schilling, G.1
  • 71
    • 0029014180 scopus 로고
    • Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals
    • Servadio, A., Koshy, B., Armstrong, D., Antalffy, B., Orr, H. T. & Zoghbi, H. Y. 1995 Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals. Nature Genet. 10, 94-98.
    • (1995) Nature Genet. , vol.10 , pp. 94-98
    • Servadio, A.1    Koshy, B.2    Armstrong, D.3    Antalffy, B.4    Orr, H.T.5    Zoghbi, H.Y.6
  • 72
    • 0029034511 scopus 로고
    • Widespread expression of Huntington's disease gene (IT15) protein product
    • Sharp, A. H. (and 15 others) 1995 Widespread expression of Huntington's disease gene (IT15) protein product. Neuron 14, 1065-1074.
    • (1995) Neuron , vol.14 , pp. 1065-1074
    • Sharp, A.H.1
  • 74
    • 0027377151 scopus 로고
    • Correlations between the onset 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. 1993 Correlations between the onset of Huntington's disease and length of the trinucleotide repeat in IT-15. Hum. Mol. Genet. 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
  • 75
    • 0029059477 scopus 로고
    • Incorporation of glutamine repeats make protein cligomerize: Implications for neurodegenerative diseases
    • Stott, K., Blackburn, J. M., Butler, P. J. G. & Perutz, M. 1995 Incorporation of glutamine repeats make protein cligomerize: implications for neurodegenerative diseases. Proc. Natl Acad. Sci. USA 92, 6509-6513.
    • (1995) Proc. Natl Acad. Sci. USA , vol.92 , pp. 6509-6513
    • Stott, K.1    Blackburn, J.M.2    Butler, P.J.G.3    Perutz, M.4
  • 80
    • 18544392423 scopus 로고    scopus 로고
    • Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila
    • Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N. & Bonini, N. M. 1998 Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell 93,939-949.
    • (1998) Cell , vol.93 , pp. 939-949
    • Warrick, J.M.1    Paulson, H.L.2    Gray-Board, G.L.3    Bui, Q.T.4    Fischbeck, K.H.5    Pittman, R.N.6    Bonini, N.M.7
  • 81
    • 0032502715 scopus 로고    scopus 로고
    • Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
    • Wellington, C. L. (and 20 others) 1998 Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J. Biol. Chem. 273, 9158-9167.
    • (1998) J. Biol. Chem. , vol.273 , pp. 9158-9167
    • Wellington, C.L.1
  • 82
    • 0030294445 scopus 로고    scopus 로고
    • The expanding world of ataxins
    • Zoghbi, H. Y. 1996 The expanding world of ataxins. Nature Genet. 14, 237-238.
    • (1996) Nature Genet. , vol.14 , pp. 237-238
    • Zoghbi, H.Y.1


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.