메뉴 건너뛰기




Volumn 24, Issue 1, 1999, Pages 275-286

Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of drpla

Author keywords

[No Author keywords available]

Indexed keywords

MUTANT PROTEIN; NUCLEAR PROTEIN;

EID: 0009744392     PISSN: 08966273     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0896-6273(00)80839-9     Document Type: Article
Times cited : (165)

References (61)
  • 2
    • 0030610409 scopus 로고    scopus 로고
    • Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees
    • Becher, M.W., Rubinsztein, D.C., Leggo, J., Wagster, M.V., Stine, O.C., Ranen, N.G., Franz, M.L., Abbott, M.H., Sherr, M., MacMillan, J.C. et al. (1997). Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees. Mov. Disord. 12, 519-530.
    • (1997) Mov. Disord. , vol.12 , pp. 519-530
    • Becher, M.W.1    Rubinsztein, D.C.2    Leggo, J.3    Wagster, M.V.4    Stine, O.C.5    Ranen, N.G.6    Franz, M.L.7    Abbott, M.H.8    Sherr, M.9    MacMillan, J.C.10
  • 3
    • 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 CAG triplet repeat length
    • Becher, M.W., Kotzuk, J.A., Sharp, A.H., Davies, S.W., Bates, G.P., Price, D.L., and Ross, C.A. (1998). Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG 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
  • 5
    • 0033030565 scopus 로고    scopus 로고
    • Evidence for proteasome involvement in polyglutamine disease: Localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro
    • Chai, Y., Koppenhafer, S.L., Shoesmith, S.J., Perez, M.K., and Paulson, H.L. (1999). Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro. Hum. Mol. Genet. 8, 673-682.
    • (1999) Hum. Mol. Genet. , vol.8 , pp. 673-682
    • Chai, Y.1    Koppenhafer, S.L.2    Shoesmith, S.J.3    Perez, M.K.4    Paulson, H.L.5
  • 7
    • 0031838352 scopus 로고    scopus 로고
    • Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1
    • Cummings, C.J., Mancini, M.A., Antalffy, B., DeFranco, D.B., Orr, H.T., and Zoghbi, H.Y. (1998). Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet. 19, 148-154.
    • (1998) Nat. Genet. , vol.19 , pp. 148-154
    • Cummings, C.J.1    Mancini, M.A.2    Antalffy, B.3    DeFranco, D.B.4    Orr, H.T.5    Zoghbi, H.Y.6
  • 9
    • 0032501740 scopus 로고    scopus 로고
    • Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions?
    • Davies, S.W., Beardsall, K., Turmaine, M., DiFiglia, M., Aronin, N., and Bates, G.P. (1998). Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet 351, 131-133.
    • (1998) Lancet , vol.351 , pp. 131-133
    • Davies, S.W.1    Beardsall, K.2    Turmaine, M.3    DiFiglia, M.4    Aronin, N.5    Bates, G.P.6
  • 10
    • 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., and 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
  • 14
    • 0029873571 scopus 로고    scopus 로고
    • Trinucleotide instability: A repeating theme in human inherited disorders
    • Gusella, J.F., and MacDonald, M.E. (1996). Trinucleotide instability: a repeating theme in human inherited disorders. Annu. Rev. Med. 47, 201-209.
    • (1996) Annu. Rev. Med. , vol.47 , pp. 201-209
    • Gusella, J.F.1    MacDonald, M.E.2
  • 17
    • 0033136692 scopus 로고    scopus 로고
    • A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
    • Hodgson, G.J., Agopyan, N., Gutekunst, C.A., Leavitt, B.R., LePiane, F., Singaraja, R., Smith, D.J., Bissada, N., McCutcheon, K., Nassir et al. (1999). A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23, 1-12.
    • (1999) Neuron , vol.23 , pp. 1-12
    • Hodgson, G.J.1    Agopyan, N.2    Gutekunst, C.A.3    Leavitt, B.R.4    LePiane, F.5    Singaraja, R.6    Smith, D.J.7    Bissada, N.8    McCutcheon, K.9    Nassir10
  • 18
    • 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 (1993). A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72, 971-983.
    • (1993) Cell , vol.72 , pp. 971-983
  • 19
    • 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., Koide, R., Shimohata, T., Yamada, M., Hayashi, Y., Takano, H., Date, H., Oyake, M., Sato, T., Sato, A. et al. (1998). Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nat. Genet. 18, 111-117.
    • (1998) Nat. Genet. , vol.18 , pp. 111-117
    • Igarashi, S.1    Koide, R.2    Shimohata, T.3    Yamada, M.4    Hayashi, Y.5    Takano, H.6    Date, H.7    Oyake, M.8    Sato, T.9    Sato, A.10
  • 20
    • 85047678013 scopus 로고
    • Dentato-rubro-pallido-luysian atrophy: A clinico-pathological study
    • Ilzuka, R., Hiramya, K., and Maehara, K., (1984). Dentato-rubro-pallido-luysian atrophy: a clinico-pathological study. J. Neurol. Neurosurg. Psychiatry 47, 1288-1298.
    • (1984) J. Neurol. Neurosurg. Psychiatry , vol.47 , pp. 1288-1298
    • Ilzuka, R.1    Hiramya, K.2    Maehara, K.3
  • 21
    • 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., and Kakizuka, A. (1996). Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nat. Genet. 13, 196-202.
    • (1996) Nat. Genet. , vol.13 , pp. 196-202
    • Ikeda, H.1    Yamaguchi, M.2    Sugai, S.3    Aze, Y.4    Narumiya, S.5    Kakizuka, A.6
  • 23
    • 0029044667 scopus 로고    scopus 로고
    • Dentatorubral-pallidoluysian atrophy: Clinical features are closely related to unstable expansions of trinucleotide (cag) repeat
    • Dentatorubral-pallidoluysian atrophy: clinical features are closely related to unstable expansions of trinucleotide (CAG) repeat. Ann. Neurol. 37, 769-775.
    • Ann. Neurol. , vol.37 , pp. 769-775
  • 24
    • 0032014092 scopus 로고    scopus 로고
    • Transglutaminase action imitates Huntington's disease: Selective polymerization of Huntingtin containing expanded polyglutamine
    • Kahlem, P., Green, H., and Djian, P. (1998). Transglutaminase action imitates Huntington's disease: selective polymerization of Huntingtin containing expanded polyglutamine. Mol. Cell. 1, 595-601.
    • (1998) Mol. Cell. , vol.1 , pp. 595-601
    • Kahlem, P.1    Green, H.2    Djian, P.3
  • 25
    • 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., and 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
  • 26
    • 0031044501 scopus 로고    scopus 로고
    • Expression and distribution of the dentatorubral-pallidoluysian atrophy gene product (atrophin-1/drplap) in neuronal and non-neuronal tissues
    • Knight, S.P., Richardson, M.M., Osmand, A.P., Stakkestad, A., and Potter, N.T. (1997). Expression and distribution of the dentatorubral-pallidoluysian atrophy gene product (atrophin-1/drplap) in neuronal and non-neuronal tissues. J. Neurol. Sci. 146, 19-26.
    • (1997) J. Neurol. Sci. , vol.146 , pp. 19-26
    • Knight, S.P.1    Richardson, M.M.2    Osmand, A.P.3    Stakkestad, A.4    Potter, N.T.5
  • 28
    • 0028815025 scopus 로고
    • DNA analysis in hereditary dentatorubral-pallidoluysian atrophy: Correlation between CAG repeat length and phenotypic variation and the molecular basis of anticipation
    • Komure, O., Sano, A., Nishino, N., Yamauchi, N., Ueno, S., Kondoh, K., Sano, N., Takahashi, M., Murayama, N., Kondo, I. et al. (1995). DNA analysis in hereditary dentatorubral-pallidoluysian atrophy: correlation between CAG repeat length and phenotypic variation and the molecular basis of anticipation. Neurology 45, 143-149.
    • (1995) Neurology , vol.45 , pp. 143-149
    • Komure, O.1    Sano, A.2    Nishino, N.3    Yamauchi, N.4    Ueno, S.5    Kondoh, K.6    Sano, N.7    Takahashi, M.8    Murayama, N.9    Kondo, I.10
  • 29
    • 0027297703 scopus 로고
    • Novel triplet repeat containing genes in human brain: Cloning, expression, and length polymorphisms
    • Li, S.H., McInnis, M.G., Margolis, R.L., Antonarakis, S.E., and Ross, C.A. (1993). Novel triplet repeat containing genes in human brain: cloning, expression, and length polymorphisms. Genomics 16, 572-579.
    • (1993) Genomics , vol.16 , pp. 572-579
    • Li, S.H.1    McInnis, M.G.2    Margolis, R.L.3    Antonarakis, S.E.4    Ross, C.A.5
  • 33
    • 0030716768 scopus 로고    scopus 로고
    • The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1
    • Matilla, A., Koshy, B.T., Cummings, C.J., Isobe, T., Orr, H.T., and Zoghbi, H.Y. (1997). The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1. Nature 389, 974-978.
    • (1997) Nature , vol.389 , pp. 974-978
    • Matilla, A.1    Koshy, B.T.2    Cummings, C.J.3    Isobe, T.4    Orr, H.T.5    Zoghbi, H.Y.6
  • 34
    • 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., and 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
  • 35
    • 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., and Yamada, M. (1998). Intracellular aggregate formation of dentatorubral-pallidoluysian atrophy (DRPLA) protein with the extended polyglutamine. Biochem. Biophys. Res. Commun. 249, 96-102.
    • (1998) Biochem. Biophys. Res. Commun. , vol.249 , pp. 96-102
    • Miyashita, T.1    Nagao, K.2    Ohmi, K.3    Yanagisawa, H.4    Okamura-Oho, Y.5    Yamada, M.6
  • 36
    • 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., and Johnson, E.M., Jr. (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
  • 37
    • 0028060244 scopus 로고
    • Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA)
    • Nagafuchi, S., Yanagisawa, H., Ohsaki, E., Shirayama, T., Tadokoro, K., Inoue, T., and Yamada, M. (1994a). Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA). Nat. Genet. 8, 177-182.
    • (1994) Nat. Genet. , vol.8 , pp. 177-182
    • Nagafuchi, S.1    Yanagisawa, H.2    Ohsaki, E.3    Shirayama, T.4    Tadokoro, K.5    Inoue, T.6    Yamada, M.7
  • 39
    • 0020064620 scopus 로고
    • Familial myoclonus epilepsy and choreoathetosis: Hereditary dentatorubral-pallidoluysian atrophy
    • Naito, H., and Oyanagi, S. (1982). Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy. Neurology 32, 798-807.
    • (1982) Neurology , vol.32 , pp. 798-807
    • Naito, H.1    Oyanagi, S.2
  • 41
    • 0029968460 scopus 로고    scopus 로고
    • Trinucleotide repeats in neurogenetic disorders
    • Paulson, H.L., and Fischbeck, K.H. (1996). Trinucleotide repeats in neurogenetic disorders. Annu. Rev. Neurosci. 19, 79-107.
    • (1996) Annu. Rev. Neurosci. , vol.19 , pp. 79-107
    • Paulson, H.L.1    Fischbeck, K.H.2
  • 42
    • 0032517816 scopus 로고    scopus 로고
    • Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation
    • Perez, M.K., Paulson, H.L., Pendse, S.J., Saionz, S.J., Bonini, N.M., and Pittman, R.N. (1998). Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. J. Cell. Biol. 143, 1457-1470.
    • (1998) J. Cell. Biol. , vol.143 , pp. 1457-1470
    • Perez, M.K.1    Paulson, H.L.2    Pendse, S.J.3    Saionz, S.J.4    Bonini, N.M.5    Pittman, R.N.6
  • 43
    • 0029401042 scopus 로고
    • Glutamine repeats as polar zippers: Their role in inherited neurodegenerative disease
    • Perutz, M.F. (1995). Glutamine repeats as polar zippers: their role in inherited neurodegenerative disease. Mol. Med. 1, 718-721.
    • (1995) Mol. Med. , vol.1 , pp. 718-721
    • Perutz, M.F.1
  • 46
    • 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
  • 47
    • 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., and Sharp, A.H. (1997a). Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology. Brain Pathol. 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
  • 49
    • 0033103523 scopus 로고    scopus 로고
    • Caspase-8 is required for cell death induced by expanded polyglutamine repeats
    • Sanchez, I., Xu, C.J., Juo, P., Kakizaka, A., Blenis, J., and 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
  • 50
    • 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., and 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
  • 54
    • 0001294843 scopus 로고
    • Unusual form of cerebellar ataxia: Combined dentato-rubral and pallido-luysian degeneration
    • Smith, J.K., Gonda, V.E., and Malamud, N. (1958). Unusual form of cerebellar ataxia: combined dentato-rubral and pallido-luysian degeneration. Neurology 8, 205-209.
    • (1958) Neurology , vol.8 , pp. 205-209
    • Smith, J.K.1    Gonda, V.E.2    Malamud, N.3
  • 55
    • 0032945938 scopus 로고    scopus 로고
    • Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
    • Stenoien, D.L., Cummings, C.J., Adams, H.P., Mancini, M.G., Patel, K., DeMartino, G.N., Marcelli, M., Weigel, N.L., and Mancini, M.A. (1999). Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum. Mol. Genet. 8, 731-741.
    • (1999) Hum. Mol. Genet. , vol.8 , pp. 731-741
    • Stenoien, D.L.1    Cummings, C.J.2    Adams, H.P.3    Mancini, M.G.4    Patel, K.5    DeMartino, G.N.6    Marcelli, M.7    Weigel, N.L.8    Mancini, M.A.9
  • 57
    • 0023680089 scopus 로고
    • Hereditary dentatorubral-pallido-luysian atrophy: Clinical and pathologic variants in a family
    • Takahashi, H., Ohama, E., Naito, H., Takeda, S., Nakashima, S., Makifuchi, T., and Ikuta, F. (1988). Hereditary dentatorubral-pallido-luysian atrophy: clinical and pathologic variants in a family. Neurology 38, 1065-1070.
    • (1988) Neurology , vol.38 , pp. 1065-1070
    • Takahashi, H.1    Ohama, E.2    Naito, H.3    Takeda, S.4    Nakashima, S.5    Makifuchi, T.6    Ikuta, F.7
  • 61
    • 0029015557 scopus 로고
    • Abnormal gene product identified in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) brain
    • Yazawa, I., Nukina, N., Hashida, H., Goto, J., Yamada, M., and Kanazawa, I. (1995). Abnormal gene product identified in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) brain. Nat. Genet. 10, 99-103.
    • (1995) Nat. Genet. , vol.10 , pp. 99-103
    • Yazawa, I.1    Nukina, N.2    Hashida, H.3    Goto, J.4    Yamada, M.5    Kanazawa, I.6


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.