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Volumn 8, Issue 8, 2012, Pages

ATXN2-CAG42 Sequesters PABPC1 into Insolubility and Induces FBXW8 in Cerebellum of Old Ataxic Knock-In Mice

Author keywords

[No Author keywords available]

Indexed keywords

ATAXIN 2; BINDING PROTEIN; MESSENGER RNA; PEPTIDES AND PROTEINS; PROTEIN PABC1; UNCLASSIFIED DRUG;

EID: 84866179163     PISSN: 15537390     EISSN: 15537404     Source Type: Journal    
DOI: 10.1371/journal.pgen.1002920     Document Type: Article
Times cited : (66)

References (69)
  • 1
    • 69949170793 scopus 로고    scopus 로고
    • The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies
    • Bauer PO, Nukina N, (2009) The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies. J Neurochem 110: 1737-1765.
    • (2009) J Neurochem , vol.110 , pp. 1737-1765
    • Bauer, P.O.1    Nukina, N.2
  • 2
    • 0030294345 scopus 로고    scopus 로고
    • Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats
    • Imbert G, Saudou F, Yvert G, Devys D, Trottier Y, et al. (1996) Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Nat Genet 14: 285-291.
    • (1996) Nat Genet , vol.14 , pp. 285-291
    • Imbert, G.1    Saudou, F.2    Yvert, G.3    Devys, D.4    Trottier, Y.5
  • 3
    • 0030292488 scopus 로고    scopus 로고
    • Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2
    • Pulst SM, Nechiporuk A, Nechiporuk T, Gispert S, Chen XN, et al. (1996) Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat Genet 14: 269-276.
    • (1996) Nat Genet , vol.14 , pp. 269-276
    • Pulst, S.M.1    Nechiporuk, A.2    Nechiporuk, T.3    Gispert, S.4    Chen, X.N.5
  • 4
    • 0030292368 scopus 로고    scopus 로고
    • Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT
    • Sanpei K, Takano H, Igarashi S, Sato T, Oyake M, et al. (1996) Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. Nat Genet 14: 277-284.
    • (1996) Nat Genet , vol.14 , pp. 277-284
    • Sanpei, K.1    Takano, H.2    Igarashi, S.3    Sato, T.4    Oyake, M.5
  • 5
    • 77956155218 scopus 로고    scopus 로고
    • Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
    • Elden AC, Kim HJ, Hart MP, Chen-Plotkin AS, Johnson BS, et al. (2010) Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Nature 466: 1069-1075.
    • (2010) Nature , vol.466 , pp. 1069-1075
    • Elden, A.C.1    Kim, H.J.2    Hart, M.P.3    Chen-Plotkin, A.S.4    Johnson, B.S.5
  • 6
    • 81955162888 scopus 로고    scopus 로고
    • The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect
    • Gispert S, Kurz A, Waibel S, Bauer P, Liepelt I, et al. (2012) The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect. Neurobiol Dis 45: 356-361.
    • (2012) Neurobiol Dis , vol.45 , pp. 356-361
    • Gispert, S.1    Kurz, A.2    Waibel, S.3    Bauer, P.4    Liepelt, I.5
  • 8
    • 79961133912 scopus 로고    scopus 로고
    • Spinocerebellar ataxia type 2
    • Auburger GW, (2012) Spinocerebellar ataxia type 2. Handb Clin Neurol 103: 423-436.
    • (2012) Handb Clin Neurol , vol.103 , pp. 423-436
    • Auburger, G.W.1
  • 9
    • 0031906658 scopus 로고    scopus 로고
    • The role of the SCA2 trinucleotide repeat expansion in 89 autosomal dominant cerebellar ataxia families. Frequency, clinical and genetic correlates
    • Giunti P, Sabbadini G, Sweeney MG, Davis MB, Veneziano L, et al. (1998) The role of the SCA2 trinucleotide repeat expansion in 89 autosomal dominant cerebellar ataxia families. Frequency, clinical and genetic correlates. Brain 121 (Pt 3):: 459-467.
    • (1998) Brain , vol.121 , Issue.Pt 3 , pp. 459-467
    • Giunti, P.1    Sabbadini, G.2    Sweeney, M.G.3    Davis, M.B.4    Veneziano, L.5
  • 10
    • 79958072622 scopus 로고    scopus 로고
    • The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study
    • Jacobi H, Bauer P, Giunti P, Labrum R, Sweeney MG, et al. (2011) The natural history of spinocerebellar ataxia type 1, 2, 3, and 6: a 2-year follow-up study. Neurology 77: 1035-1041.
    • (2011) Neurology , vol.77 , pp. 1035-1041
    • Jacobi, H.1    Bauer, P.2    Giunti, P.3    Labrum, R.4    Sweeney, M.G.5
  • 12
    • 1242338792 scopus 로고    scopus 로고
    • Assessment of the nutrition status of patients with Huntington's disease
    • Trejo A, Tarrats RM, Alonso ME, Boll MC, Ochoa A, et al. (2004) Assessment of the nutrition status of patients with Huntington's disease. Nutrition 20: 192-196.
    • (2004) Nutrition , vol.20 , pp. 192-196
    • Trejo, A.1    Tarrats, R.M.2    Alonso, M.E.3    Boll, M.C.4    Ochoa, A.5
  • 14
    • 41149108443 scopus 로고    scopus 로고
    • Spinocerebellar ataxia type 2 (SCA2) in an Egyptian family presenting with polyphagia and marked CAG expansion in infancy
    • Abdel-Aleem A, Zaki MS, (2008) Spinocerebellar ataxia type 2 (SCA2) in an Egyptian family presenting with polyphagia and marked CAG expansion in infancy. J Neurol 255: 413-419.
    • (2008) J Neurol , vol.255 , pp. 413-419
    • Abdel-Aleem, A.1    Zaki, M.S.2
  • 16
    • 22144453212 scopus 로고    scopus 로고
    • Involvement of the cranial nerves and their nuclei in spinocerebellar ataxia type 2 (SCA2)
    • Gierga K, Burk K, Bauer M, Orozco Diaz G, Auburger G, et al. (2005) Involvement of the cranial nerves and their nuclei in spinocerebellar ataxia type 2 (SCA2). Acta Neuropathol 109: 617-631.
    • (2005) Acta Neuropathol , vol.109 , pp. 617-631
    • Gierga, K.1    Burk, K.2    Bauer, M.3    Orozco Diaz, G.4    Auburger, G.5
  • 17
    • 52949135556 scopus 로고    scopus 로고
    • Involvement of the auditory brainstem system in spinocerebellar ataxia type 2 (SCA2), type 3 (SCA3) and type 7 (SCA7)
    • Hoche F, Seidel K, Brunt ER, Auburger G, Schols L, et al. (2008) Involvement of the auditory brainstem system in spinocerebellar ataxia type 2 (SCA2), type 3 (SCA3) and type 7 (SCA7). Neuropathol Appl Neurobiol 34: 479-491.
    • (2008) Neuropathol Appl Neurobiol , vol.34 , pp. 479-491
    • Hoche, F.1    Seidel, K.2    Brunt, E.R.3    Auburger, G.4    Schols, L.5
  • 18
    • 0024997225 scopus 로고
    • Autosomal dominant cerebellar ataxia: clinical analysis of 263 patients from a homogeneous population in Holguin, Cuba
    • Orozco Diaz G, Nodarse Fleites A, Cordoves Sagaz R, Auburger G, (1990) Autosomal dominant cerebellar ataxia: clinical analysis of 263 patients from a homogeneous population in Holguin, Cuba. Neurology 40: 1369-1375.
    • (1990) Neurology , vol.40 , pp. 1369-1375
    • Orozco Diaz, G.1    Nodarse Fleites, A.2    Cordoves Sagaz, R.3    Auburger, G.4
  • 19
    • 17044447789 scopus 로고    scopus 로고
    • Anatomically based guidelines for systematic investigation of the central somatosensory system and their application to a spinocerebellar ataxia type 2 (SCA2) patient
    • Rub U, Schultz C, Del Tredici K, Gierga K, Reifenberger G, et al. (2003) Anatomically based guidelines for systematic investigation of the central somatosensory system and their application to a spinocerebellar ataxia type 2 (SCA2) patient. Neuropathol Appl Neurobiol 29: 418-433.
    • (2003) Neuropathol Appl Neurobiol , vol.29 , pp. 418-433
    • Rub, U.1    Schultz, C.2    Del Tredici, K.3    Gierga, K.4    Reifenberger, G.5
  • 20
    • 33846635034 scopus 로고    scopus 로고
    • Consistent affection of the central somatosensory system in spinocerebellar ataxia type 2 and type 3 and its significance for clinical symptoms and rehabilitative therapy
    • Rub U, Seidel K, Ozerden I, Gierga K, Brunt ER, et al. (2007) Consistent affection of the central somatosensory system in spinocerebellar ataxia type 2 and type 3 and its significance for clinical symptoms and rehabilitative therapy. Brain Res Rev 53: 235-249.
    • (2007) Brain Res Rev , vol.53 , pp. 235-249
    • Rub, U.1    Seidel, K.2    Ozerden, I.3    Gierga, K.4    Brunt, E.R.5
  • 21
    • 84872670701 scopus 로고    scopus 로고
    • Pathoanatomy of Cerebellar Degeneration in Spinocerebellar Ataxia Type 2 (SCA2) and Type 3 (SCA3)
    • Scherzed W, Brunt ER, Heinsen H, de Vos RA, Seidel K, et al. (2011) Pathoanatomy of Cerebellar Degeneration in Spinocerebellar Ataxia Type 2 (SCA2) and Type 3 (SCA3). Cerebellum.
    • (2011) Cerebellum
    • Scherzed, W.1    Brunt, E.R.2    Heinsen, H.3    de Vos, R.A.4    Seidel, K.5
  • 22
    • 33845697708 scopus 로고    scopus 로고
    • Stages of sleep pathology in spinocerebellar ataxia type 2 (SCA2)
    • Tuin I, Voss U, Kang JS, Kessler K, Rub U, et al. (2006) Stages of sleep pathology in spinocerebellar ataxia type 2 (SCA2). Neurology 67: 1966-1972.
    • (2006) Neurology , vol.67 , pp. 1966-1972
    • Tuin, I.1    Voss, U.2    Kang, J.S.3    Kessler, K.4    Rub, U.5
  • 23
    • 4444330529 scopus 로고    scopus 로고
    • Saccade velocity is controlled by polyglutamine size in spinocerebellar ataxia 2
    • Velazquez-Perez L, Seifried C, Santos-Falcon N, Abele M, Ziemann U, et al. (2004) Saccade velocity is controlled by polyglutamine size in spinocerebellar ataxia 2. Ann Neurol 56: 444-447.
    • (2004) Ann Neurol , vol.56 , pp. 444-447
    • Velazquez-Perez, L.1    Seifried, C.2    Santos-Falcon, N.3    Abele, M.4    Ziemann, U.5
  • 24
    • 36349021396 scopus 로고    scopus 로고
    • Are interrupted SCA2 CAG repeat expansions responsible for parkinsonism?
    • Charles P, Camuzat A, Benammar N, Sellal F, Destee A, et al. (2007) Are interrupted SCA2 CAG repeat expansions responsible for parkinsonism? Neurology 69: 1970-1975.
    • (2007) Neurology , vol.69 , pp. 1970-1975
    • Charles, P.1    Camuzat, A.2    Benammar, N.3    Sellal, F.4    Destee, A.5
  • 25
    • 0034718577 scopus 로고    scopus 로고
    • Spinocerebellar ataxia type 2 with parkinsonism in ethnic Chinese
    • Gwinn-Hardy K, Chen JY, Liu HC, Liu TY, Boss M, et al. (2000) Spinocerebellar ataxia type 2 with parkinsonism in ethnic Chinese. Neurology 55: 800-805.
    • (2000) Neurology , vol.55 , pp. 800-805
    • Gwinn-Hardy, K.1    Chen, J.Y.2    Liu, H.C.3    Liu, T.Y.4    Boss, M.5
  • 26
    • 27144467422 scopus 로고    scopus 로고
    • Spinocerebellar ataxias types 2 and 3: degeneration of the pre-cerebellar nuclei isolates the three phylogenetically defined regions of the cerebellum
    • Rub U, Gierga K, Brunt ER, de Vos RA, Bauer M, et al. (2005) Spinocerebellar ataxias types 2 and 3: degeneration of the pre-cerebellar nuclei isolates the three phylogenetically defined regions of the cerebellum. J Neural Transm 112: 1523-1545.
    • (2005) J Neural Transm , vol.112 , pp. 1523-1545
    • Rub, U.1    Gierga, K.2    Brunt, E.R.3    de Vos, R.A.4    Bauer, M.5
  • 27
    • 80051779924 scopus 로고    scopus 로고
    • Spinocerebellar ataxia type 2 (SCA2): identification of early brain degeneration in one monozygous twin in the initial disease stage
    • Hoche F, Baliko L, den Dunnen W, Steinecker K, Bartos L, et al. (2011) Spinocerebellar ataxia type 2 (SCA2): identification of early brain degeneration in one monozygous twin in the initial disease stage. Cerebellum 10: 245-253.
    • (2011) Cerebellum , vol.10 , pp. 245-253
    • Hoche, F.1    Baliko, L.2    den Dunnen, W.3    Steinecker, K.4    Bartos, L.5
  • 28
    • 9144221463 scopus 로고    scopus 로고
    • Complex phenotypes in an Indian family with homozygous SCA2 mutations
    • Ragothaman M, Sarangmath N, Chaudhary S, Khare V, Mittal U, et al. (2004) Complex phenotypes in an Indian family with homozygous SCA2 mutations. Ann Neurol 55: 130-133.
    • (2004) Ann Neurol , vol.55 , pp. 130-133
    • Ragothaman, M.1    Sarangmath, N.2    Chaudhary, S.3    Khare, V.4    Mittal, U.5
  • 29
    • 0032516918 scopus 로고    scopus 로고
    • Phenotype variation correlates with CAG repeat length in SCA2-a study of 28 Japanese patients
    • Sasaki H, Wakisaka A, Sanpei K, Takano H, Igarashi S, et al. (1998) Phenotype variation correlates with CAG repeat length in SCA2-a study of 28 Japanese patients. J Neurol Sci 159: 202-208.
    • (1998) J Neurol Sci , vol.159 , pp. 202-208
    • Sasaki, H.1    Wakisaka, A.2    Sanpei, K.3    Takano, H.4    Igarashi, S.5
  • 30
    • 34249060910 scopus 로고    scopus 로고
    • Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor-A unusual clinical benefit
    • Freund HJ, Barnikol UB, Nolte D, Treuer H, Auburger G, et al. (2007) Subthalamic-thalamic DBS in a case with spinocerebellar ataxia type 2 and severe tremor-A unusual clinical benefit. Mov Disord 22: 732-735.
    • (2007) Mov Disord , vol.22 , pp. 732-735
    • Freund, H.J.1    Barnikol, U.B.2    Nolte, D.3    Treuer, H.4    Auburger, G.5
  • 31
    • 0033811788 scopus 로고    scopus 로고
    • Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human
    • Huynh DP, Figueroa K, Hoang N, Pulst SM, (2000) Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Nat Genet 26: 44-50.
    • (2000) Nat Genet , vol.26 , pp. 44-50
    • Huynh, D.P.1    Figueroa, K.2    Hoang, N.3    Pulst, S.M.4
  • 32
    • 0036786139 scopus 로고    scopus 로고
    • Paradoxical absence of nuclear inclusion in cerebellar Purkinje cells of hereditary ataxias linked to CAG expansion
    • Koyano S, Iwabuchi K, Yagishita S, Kuroiwa Y, Uchihara T, (2002) Paradoxical absence of nuclear inclusion in cerebellar Purkinje cells of hereditary ataxias linked to CAG expansion. J Neurol Neurosurg Psychiatry 73: 450-452.
    • (2002) J Neurol Neurosurg Psychiatry , vol.73 , pp. 450-452
    • Koyano, S.1    Iwabuchi, K.2    Yagishita, S.3    Kuroiwa, Y.4    Uchihara, T.5
  • 33
    • 0036185711 scopus 로고    scopus 로고
    • Neuronal intranuclear inclusions in SCA2: a genetic, morphological and immunohistochemical study of two cases
    • Pang JT, Giunti P, Chamberlain S, An SF, Vitaliani R, et al. (2002) Neuronal intranuclear inclusions in SCA2: a genetic, morphological and immunohistochemical study of two cases. Brain 125: 656-663.
    • (2002) Brain , vol.125 , pp. 656-663
    • Pang, J.T.1    Giunti, P.2    Chamberlain, S.3    An, S.F.4    Vitaliani, R.5
  • 34
    • 34547692622 scopus 로고    scopus 로고
    • Trinucleotide repeat disorders
    • Orr HT, Zoghbi HY, (2007) Trinucleotide repeat disorders. Annu Rev Neurosci 30: 575-621.
    • (2007) Annu Rev Neurosci , vol.30 , pp. 575-621
    • Orr, H.T.1    Zoghbi, H.Y.2
  • 35
    • 80555125089 scopus 로고    scopus 로고
    • Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua
    • Fryer JD, Yu P, Kang H, Mandel-Brehm C, Carter AN, et al. (2011) Exercise and genetic rescue of SCA1 via the transcriptional repressor Capicua. Science 334: 690-693.
    • (2011) Science , vol.334 , pp. 690-693
    • Fryer, J.D.1    Yu, P.2    Kang, H.3    Mandel-Brehm, C.4    Carter, A.N.5
  • 36
    • 0037101834 scopus 로고    scopus 로고
    • Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor
    • Lieberman AP, Harmison G, Strand AD, Olson JM, Fischbeck KH, (2002) Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor. Hum Mol Genet 11: 1967-1976.
    • (2002) Hum Mol Genet , vol.11 , pp. 1967-1976
    • Lieberman, A.P.1    Harmison, G.2    Strand, A.D.3    Olson, J.M.4    Fischbeck, K.H.5
  • 37
    • 77955643169 scopus 로고    scopus 로고
    • Molecular mechanisms and potential therapeutical targets in Huntington's disease
    • Zuccato C, Valenza M, Cattaneo E, (2010) Molecular mechanisms and potential therapeutical targets in Huntington's disease. Physiol Rev 90: 905-981.
    • (2010) Physiol Rev , vol.90 , pp. 905-981
    • Zuccato, C.1    Valenza, M.2    Cattaneo, E.3
  • 38
    • 49549111127 scopus 로고    scopus 로고
    • Ataxin-2 associates with the endocytosis complex and affects EGF receptor trafficking
    • Nonis D, Schmidt MH, van de Loo S, Eich F, Dikic I, et al. (2008) Ataxin-2 associates with the endocytosis complex and affects EGF receptor trafficking. Cell Signal 20: 1725-1739.
    • (2008) Cell Signal , vol.20 , pp. 1725-1739
    • Nonis, D.1    Schmidt, M.H.2    van de Loo, S.3    Eich, F.4    Dikic, I.5
  • 39
    • 12344317072 scopus 로고    scopus 로고
    • An integrative approach to gain insights into the cellular function of human ataxin-2
    • Ralser M, Albrecht M, Nonhoff U, Lengauer T, Lehrach H, et al. (2005) An integrative approach to gain insights into the cellular function of human ataxin-2. J Mol Biol 346: 203-214.
    • (2005) J Mol Biol , vol.346 , pp. 203-214
    • Ralser, M.1    Albrecht, M.2    Nonhoff, U.3    Lengauer, T.4    Lehrach, H.5
  • 40
    • 33747884761 scopus 로고    scopus 로고
    • Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes
    • Satterfield TF, Pallanck LJ, (2006) Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes. Hum Mol Genet 15: 2523-2532.
    • (2006) Hum Mol Genet , vol.15 , pp. 2523-2532
    • Satterfield, T.F.1    Pallanck, L.J.2
  • 41
    • 34247229733 scopus 로고    scopus 로고
    • Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules
    • Nonhoff U, Ralser M, Welzel F, Piccini I, Balzereit D, et al. (2007) Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules. Mol Biol Cell 18: 1385-1396.
    • (2007) Mol Biol Cell , vol.18 , pp. 1385-1396
    • Nonhoff, U.1    Ralser, M.2    Welzel, F.3    Piccini, I.4    Balzereit, D.5
  • 43
    • 0033044001 scopus 로고    scopus 로고
    • Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2
    • Huynh DP, Del Bigio MR, Ho DH, Pulst SM, (1999) Expression of ataxin-2 in brains from normal individuals and patients with Alzheimer's disease and spinocerebellar ataxia 2. Ann Neurol 45: 232-241.
    • (1999) Ann Neurol , vol.45 , pp. 232-241
    • Huynh, D.P.1    Del Bigio, M.R.2    Ho, D.H.3    Pulst, S.M.4
  • 44
    • 0037379663 scopus 로고    scopus 로고
    • Modelling brain diseases in mice: the challenges of design and analysis
    • Watase K, Zoghbi HY, (2003) Modelling brain diseases in mice: the challenges of design and analysis. Nat Rev Genet 4: 296-307.
    • (2003) Nat Rev Genet , vol.4 , pp. 296-307
    • Watase, K.1    Zoghbi, H.Y.2
  • 45
    • 0028178522 scopus 로고
    • The N-terminal domain of the human TATA-binding protein plays a role in transcription from TATA-containing RNA polymerase II and III promoters
    • Lescure A, Lutz Y, Eberhard D, Jacq X, Krol A, et al. (1994) The N-terminal domain of the human TATA-binding protein plays a role in transcription from TATA-containing RNA polymerase II and III promoters. Embo J 13: 1166-1175.
    • (1994) Embo J , vol.13 , pp. 1166-1175
    • Lescure, A.1    Lutz, Y.2    Eberhard, D.3    Jacq, X.4    Krol, A.5
  • 48
    • 4544250782 scopus 로고    scopus 로고
    • Possible function of the ADAM1a/ADAM2 Fertilin complex in the appearance of ADAM3 on the sperm surface
    • Nishimura H, Kim E, Nakanishi T, Baba T, (2004) Possible function of the ADAM1a/ADAM2 Fertilin complex in the appearance of ADAM3 on the sperm surface. J Biol Chem 279: 34957-34962.
    • (2004) J Biol Chem , vol.279 , pp. 34957-34962
    • Nishimura, H.1    Kim, E.2    Nakanishi, T.3    Baba, T.4
  • 50
    • 79955775363 scopus 로고    scopus 로고
    • Depletion of cellular poly (A) binding protein prevents protein synthesis and leads to apoptosis in HeLa cells
    • Thangima Zannat M, Bhattacharjee RB, Bag J, (2011) Depletion of cellular poly (A) binding protein prevents protein synthesis and leads to apoptosis in HeLa cells. Biochem Biophys Res Commun 408: 375-381.
    • (2011) Biochem Biophys Res Commun , vol.408 , pp. 375-381
    • Thangima Zannat, M.1    Bhattacharjee, R.B.2    Bag, J.3
  • 51
    • 58149330117 scopus 로고    scopus 로고
    • General RNA-binding proteins have a function in poly(A)-binding protein-dependent translation
    • Svitkin YV, Evdokimova VM, Brasey A, Pestova TV, Fantus D, et al. (2009) General RNA-binding proteins have a function in poly(A)-binding protein-dependent translation. Embo J 28: 58-68.
    • (2009) Embo J , vol.28 , pp. 58-68
    • Svitkin, Y.V.1    Evdokimova, V.M.2    Brasey, A.3    Pestova, T.V.4    Fantus, D.5
  • 52
    • 38449123517 scopus 로고    scopus 로고
    • Mammalian stress granules and processing bodies
    • Kedersha N, Anderson P, (2007) Mammalian stress granules and processing bodies. Methods Enzymol 431: 61-81.
    • (2007) Methods Enzymol , vol.431 , pp. 61-81
    • Kedersha, N.1    Anderson, P.2
  • 53
    • 45449118785 scopus 로고    scopus 로고
    • Recognition of nonsense mRNA: towards a unified model
    • Muhlemann O, (2008) Recognition of nonsense mRNA: towards a unified model. Biochem Soc Trans 36: 497-501.
    • (2008) Biochem Soc Trans , vol.36 , pp. 497-501
    • Muhlemann, O.1
  • 54
    • 79959726422 scopus 로고    scopus 로고
    • The splicing regulator Rbfox1 (A2BP1) controls neuronal excitation in the mammalian brain
    • Gehman LT, Stoilov P, Maguire J, Damianov A, Lin CH, et al. (2011) The splicing regulator Rbfox1 (A2BP1) controls neuronal excitation in the mammalian brain. Nat Genet 43: 706-711.
    • (2011) Nat Genet , vol.43 , pp. 706-711
    • Gehman, L.T.1    Stoilov, P.2    Maguire, J.3    Damianov, A.4    Lin, C.H.5
  • 55
    • 27644455141 scopus 로고    scopus 로고
    • Homologues of the Caenorhabditis elegans Fox-1 protein are neuronal splicing regulators in mammals
    • Underwood JG, Boutz PL, Dougherty JD, Stoilov P, Black DL, (2005) Homologues of the Caenorhabditis elegans Fox-1 protein are neuronal splicing regulators in mammals. Mol Cell Biol 25: 10005-10016.
    • (2005) Mol Cell Biol , vol.25 , pp. 10005-10016
    • Underwood, J.G.1    Boutz, P.L.2    Dougherty, J.D.3    Stoilov, P.4    Black, D.L.5
  • 56
    • 79956301405 scopus 로고    scopus 로고
    • The KRAB-containing zinc-finger transcriptional regulator ZBRK1 activates SCA2 gene transcription through direct interaction with its gene product, ataxin-2
    • Hallen L, Klein H, Stoschek C, Wehrmeyer S, Nonhoff U, et al. (2011) The KRAB-containing zinc-finger transcriptional regulator ZBRK1 activates SCA2 gene transcription through direct interaction with its gene product, ataxin-2. Hum Mol Genet 20: 104-114.
    • (2011) Hum Mol Genet , vol.20 , pp. 104-114
    • Hallen, L.1    Klein, H.2    Stoschek, C.3    Wehrmeyer, S.4    Nonhoff, U.5
  • 57
    • 79953176451 scopus 로고    scopus 로고
    • Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients
    • Lee T, Li YR, Ingre C, Weber M, Grehl T, et al. (2011) Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients. Hum Mol Genet 20: 1697-1700.
    • (2011) Hum Mol Genet , vol.20 , pp. 1697-1700
    • Lee, T.1    Li, Y.R.2    Ingre, C.3    Weber, M.4    Grehl, T.5
  • 59
    • 33745592187 scopus 로고    scopus 로고
    • Loss of endogenous androgen receptor protein accelerates motor neuron degeneration and accentuates androgen insensitivity in a mouse model of X-linked spinal and bulbar muscular atrophy
    • Thomas PS Jr, Fraley GS, Damian V, Woodke LB, Zapata F, et al. (2006) Loss of endogenous androgen receptor protein accelerates motor neuron degeneration and accentuates androgen insensitivity in a mouse model of X-linked spinal and bulbar muscular atrophy. Hum Mol Genet 15: 2225-2238.
    • (2006) Hum Mol Genet , vol.15 , pp. 2225-2238
    • Thomas Jr., P.S.1    Fraley, G.S.2    Damian, V.3    Woodke, L.B.4    Zapata, F.5
  • 60
    • 8144228406 scopus 로고    scopus 로고
    • Trinucleotide repeats and neurodegenerative disease
    • Everett CM, Wood NW, (2004) Trinucleotide repeats and neurodegenerative disease. Brain 127: 2385-2405.
    • (2004) Brain , vol.127 , pp. 2385-2405
    • Everett, C.M.1    Wood, N.W.2
  • 61
    • 78650101277 scopus 로고    scopus 로고
    • Histone acetylation, acetyltransferases, and ataxia-alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disorders
    • McCullough SD, Grant PA, (2010) Histone acetylation, acetyltransferases, and ataxia-alteration of histone acetylation and chromatin dynamics is implicated in the pathogenesis of polyglutamine-expansion disorders. Adv Protein Chem Struct Biol 79: 165-203.
    • (2010) Adv Protein Chem Struct Biol , vol.79 , pp. 165-203
    • McCullough, S.D.1    Grant, P.A.2
  • 62
    • 37849020693 scopus 로고    scopus 로고
    • Disruption of the Fbxw8 gene results in pre- and postnatal growth retardation in mice
    • Tsutsumi T, Kuwabara H, Arai T, Xiao Y, Decaprio JA, (2008) Disruption of the Fbxw8 gene results in pre- and postnatal growth retardation in mice. Mol Cell Biol 28: 743-751.
    • (2008) Mol Cell Biol , vol.28 , pp. 743-751
    • Tsutsumi, T.1    Kuwabara, H.2    Arai, T.3    Xiao, Y.4    Decaprio, J.A.5
  • 63
    • 79958046350 scopus 로고    scopus 로고
    • An OBSL1-Cul7Fbxw8 ubiquitin ligase signaling mechanism regulates Golgi morphology and dendrite patterning
    • Litterman N, Ikeuchi Y, Gallardo G, O'Connell BC, Sowa ME, et al. (2011) An OBSL1-Cul7Fbxw8 ubiquitin ligase signaling mechanism regulates Golgi morphology and dendrite patterning. PLoS Biol 9: e1001060 doi:10.1371/journal.pbio.1001060.
    • (2011) PLoS Biol , vol.9
    • Litterman, N.1    Ikeuchi, Y.2    Gallardo, G.3    O'Connell, B.C.4    Sowa, M.E.5
  • 64
    • 33846545106 scopus 로고    scopus 로고
    • Parkin is an E3 ubiquitin-ligase for normal and mutant ataxin-2 and prevents ataxin-2-induced cell death
    • Huynh DP, Nguyen DT, Pulst-Korenberg JB, Brice A, Pulst SM, (2007) Parkin is an E3 ubiquitin-ligase for normal and mutant ataxin-2 and prevents ataxin-2-induced cell death. Exp Neurol 203: 531-541.
    • (2007) Exp Neurol , vol.203 , pp. 531-541
    • Huynh, D.P.1    Nguyen, D.T.2    Pulst-Korenberg, J.B.3    Brice, A.4    Pulst, S.M.5
  • 65
    • 77956147372 scopus 로고    scopus 로고
    • Neurodegeneration: An expansion in ALS genetics
    • Lagier-Tourenne C, Cleveland DW, (2010) Neurodegeneration: An expansion in ALS genetics. Nature 466: 1052-1053.
    • (2010) Nature , vol.466 , pp. 1052-1053
    • Lagier-Tourenne, C.1    Cleveland, D.W.2
  • 66
    • 37749039120 scopus 로고    scopus 로고
    • dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1
    • Al-Ramahi I, Perez AM, Lim J, Zhang M, Sorensen R, et al. (2007) dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet 3: e234 doi:10.1371/journal.pgen.0030234.
    • (2007) PLoS Genet , vol.3
    • Al-Ramahi, I.1    Perez, A.M.2    Lim, J.3    Zhang, M.4    Sorensen, R.5
  • 67
    • 0030864463 scopus 로고    scopus 로고
    • Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations
    • Clark HB, Burright EN, Yunis WS, Larson S, Wilcox C, et al. (1997) Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. J Neurosci 17: 7385-7395.
    • (1997) J Neurosci , vol.17 , pp. 7385-7395
    • Clark, H.B.1    Burright, E.N.2    Yunis, W.S.3    Larson, S.4    Wilcox, C.5
  • 68
    • 0035710746 scopus 로고    scopus 로고
    • Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method
    • Livak KJ, Schmittgen TD, (2001) Analysis of relative gene expression data using real-time quantitative PCR and the 2(-Delta Delta C(T)) Method. Methods 25: 402-408.
    • (2001) Methods , vol.25 , pp. 402-408
    • Livak, K.J.1    Schmittgen, T.D.2
  • 69
    • 0036081355 scopus 로고    scopus 로고
    • Gene Expression Omnibus: NCBI gene expression and hybridization array data repository
    • Edgar R, Domrachev M, Lash AE, (2002) Gene Expression Omnibus: NCBI gene expression and hybridization array data repository. Nucleic Acids Res 30: 207-210.
    • (2002) Nucleic Acids Res , vol.30 , pp. 207-210
    • Edgar, R.1    Domrachev, M.2    Lash, A.E.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.