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Human Molecular Genetics, 2000, Vol. 9, No. 14 2197-2202
© 2000 Oxford University Press

CREB-binding protein sequestration by expanded polyglutamine

Alexander McCampbell+, J. Paul Taylor, Addis A. Taye, Jon Robitschek, Mei Li1, Jessica Walcott2, Diane Merry2, Yaohui Chai3, Henry Paulson3, Gen Sobue1 and Kenneth H. Fischbeck

Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 3B11, Bethesda, MD 20892-1250, USA, 1Department of Neurology, Nagoya University School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi, Japan, 2208 Blumle Life Science Building, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA and 3Department of Neurology, University of Iowa College of Medicine, 2007 RCP, Iowa City, IA 52242, USA

Spinal and bulbar muscular atrophy (SBMA) is one of eight inherited neurodegenerative diseases known to be caused by CAG repeat expansion. The expansion results in an expanded polyglutamine tract, which likely confers a novel, toxic function to the affected protein. Cell culture and transgenic mouse studies have implicated the nucleus as a site for pathogenesis, suggesting that a critical nuclear factor or process is disrupted by the polyglutamine expansion. In this report we present evidence that CREB-binding protein (CBP), a transcriptional co-activator that orchestrates nuclear response to a variety of cell signaling cascades, is incorporated into nuclear inclusions formed by polyglutamine-containing proteins in cultured cells, transgenic mice and tissue from patients with SBMA. We also show CBP incorporation into nuclear inclusions formed in a cell culture model of another polyglutamine disease, spinocerebellar ataxia type 3. We present evidence that soluble levels of CBP are reduced in cells expressing expanded polyglutamine despite increased levels of CBP mRNA. Finally, we demonstrate that over-expression of CBP rescues cells from polyglutamine-mediated toxicity in neuronal cell culture. These data support a CBP-sequestration model of polyglutamine expansion disease.

+ To whom correspondence should be addressed. Tel: +1 301 435 9288; Fax: +1 301 480 3365; Email: mccampba@ninds.nih.gov


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Home page
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[Abstract] [Full Text] [PDF]


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J. Neurosci.Home page
M. Katsuno, H. Adachi, M. Minamiyama, M. Waza, K. Tokui, H. Banno, K. Suzuki, Y. Onoda, F. Tanaka, M. Doyu, et al.
Reversible Disruption of Dynactin 1-Mediated Retrograde Axonal Transport in Polyglutamine-Induced Motor Neuron Degeneration.
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[Abstract] [Full Text] [PDF]


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[Abstract] [Full Text] [PDF]


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Progressive Disruption of Cellular Protein Folding in Models of Polyglutamine Diseases
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Home page
J. Neurosci.Home page
E. A. Bates, M. Victor, A. K. Jones, Y. Shi, and A. C. Hart
Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity.
J. Neurosci., March 8, 2006; 26(10): 2830 - 2838.
[Abstract] [Full Text] [PDF]


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PNAS, July 19, 2005; 102(29): 10261 - 10266.
[Abstract] [Full Text] [PDF]


Home page
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M. Thomas, Z. Yu, N. Dadgar, S. Varambally, J. Yu, A. M. Chinnaiyan, and A. P. Lieberman
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J. Biol. Chem., June 3, 2005; 280(22): 21264 - 21271.
[Abstract] [Full Text] [PDF]


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[Abstract] [Full Text] [PDF]


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[Abstract] [PDF]


Home page
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Hsp70 dynamics in vivo: effect of heat shock and protein aggregation
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[Abstract] [Full Text] [PDF]


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J. Biol. Chem., February 27, 2004; 279(9): 8389 - 8395.
[Abstract] [Full Text] [PDF]


Home page
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J. Biol. Chem., February 6, 2004; 279(6): 4988 - 4999.
[Abstract] [Full Text] [PDF]


Home page
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Y. Chai, S. S. Berke, R. E. Cohen, and H. L. Paulson
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J. Biol. Chem., January 30, 2004; 279(5): 3605 - 3611.
[Abstract] [Full Text] [PDF]


Home page
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[Abstract] [Full Text] [PDF]


Home page
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Hum. Mol. Genet., January 1, 2004; 13(1): 53 - 67.
[Abstract] [Full Text] [PDF]


Home page
J. Neurosci.Home page
R. J. Ferrante, J. K. Kubilus, J. Lee, H. Ryu, A. Beesen, B. Zucker, K. Smith, N. W. Kowall, R. R. Ratan, R. Luthi-Carter, et al.
Histone Deacetylase Inhibition by Sodium Butyrate Chemotherapy Ameliorates the Neurodegenerative Phenotype in Huntington's Disease Mice
J. Neurosci., October 15, 2003; 23(28): 9418 - 9427.
[Abstract] [Full Text] [PDF]


Home page
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A. Michalik and C. Van Broeckhoven
Pathogenesis of polyglutamine disorders: aggregation revisited
Hum. Mol. Genet., October 15, 2003; 12(90002): R173 - 186.
[Abstract] [Full Text] [PDF]


Home page
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Hum. Mol. Genet., July 1, 2003; 12(13): 1535 - 1542.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
K. Ishihara, N. Yamagishi, Y. Saito, H. Adachi, Y. Kobayashi, G. Sobue, K. Ohtsuka, and T. Hatayama
Hsp105{alpha} Suppresses the Aggregation of Truncated Androgen Receptor with Expanded CAG Repeats and Cell Toxicity
J. Biol. Chem., June 27, 2003; 278(27): 25143 - 25150.
[Abstract] [Full Text] [PDF]


Home page
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J. P. Taylor, A. A. Taye, C. Campbell, P. Kazemi-Esfarjani, K. H. Fischbeck, and K.-T. Min
Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein
Genes & Dev., June 15, 2003; 17(12): 1463 - 1468.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
Y. C. Tsai, P. S. Fishman, N. V. Thakor, and G. A. Oyler
Parkin Facilitates the Elimination of Expanded Polyglutamine Proteins and Leads to Preservation of Proteasome Function
J. Biol. Chem., June 6, 2003; 278(24): 22044 - 22055.
[Abstract] [Full Text] [PDF]


Home page
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Prevention of polyglutamine oligomerization and neurodegeneration by the peptide inhibitor QBP1 in Drosophila
Hum. Mol. Genet., June 1, 2003; 12(11): 1253 - 1259.
[Abstract] [Full Text] [PDF]


Home page
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Hum. Mol. Genet., April 1, 2003; 12(7): 711 - 725.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
J. P. Taylor, F. Tanaka, J. Robitschek, C. M. Sandoval, A. Taye, S. Markovic-Plese, and K. H. Fischbeck
Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein
Hum. Mol. Genet., April 1, 2003; 12(7): 749 - 757.
[Abstract] [Full Text] [PDF]


Home page
J. Neurosci.Home page
H. Adachi, M. Katsuno, M. Minamiyama, C. Sang, G. Pagoulatos, C. Angelidis, M. Kusakabe, A. Yoshiki, Y. Kobayashi, M. Doyu, et al.
Heat Shock Protein 70 Chaperone Overexpression Ameliorates Phenotypes of the Spinal and Bulbar Muscular Atrophy Transgenic Mouse Model by Reducing Nuclear-Localized Mutant Androgen Receptor Protein
J. Neurosci., March 15, 2003; 23(6): 2203 - 2211.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
S. Gines, I. S. Seong, E. Fossale, E. Ivanova, F. Trettel, J. F. Gusella, V. C. Wheeler, F. Persichetti, and M. E. MacDonald
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice
Hum. Mol. Genet., March 1, 2003; 12(5): 497 - 508.
[Abstract] [Full Text] [PDF]


Home page
Proc. Natl. Acad. Sci. USAHome page
C. A. Ross, M. A. Poirier, E. E. Wanker, and M. Amzel
Polyglutamine fibrillogenesis: The pathway unfolds
PNAS, January 7, 2003; 100(1): 1 - 3.
[Full Text] [PDF]


Home page
Hum Mol GenetHome page
H. Jiang, F. C. Nucifora Jr, C. A. Ross, and D. B. DeFranco
Cell death triggered by polyglutamine-expanded huntingtin in a neuronal cell line is associated with degradation of CREB-binding protein
Hum. Mol. Genet., January 1, 2003; 12(1): 1 - 12.
[Abstract] [Full Text] [PDF]


Home page
Proc. Natl. Acad. Sci. USAHome page
P. W. Faber, C. Voisine, D. C. King, E. A. Bates, and A. C. Hart
Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity
PNAS, December 24, 2002; 99(26): 17131 - 17136.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
F. Li, T. Macfarlan, R. N. Pittman, and D. Chakravarti
Ataxin-3 Is a Histone-binding Protein with Two Independent Transcriptional Corepressor Activities
J. Biol. Chem., November 15, 2002; 277(47): 45004 - 45012.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
P. Kazemi-Esfarjani and S. Benzer
Suppression of polyglutamine toxicity by a Drosophila homolog of myeloid leukemia factor 1
Hum. Mol. Genet., October 2, 2002; 11(21): 2657 - 2672.
[Abstract] [Full Text] [PDF]


Home page
J. Biol. Chem.Home page
Y. Kimura, S. Koitabashi, A. Kakizuka, and T. Fujita
Circumvention of Chaperone Requirement for Aggregate Formation of a Short Polyglutamine Tract by the Co-expression of a Long Polyglutamine Tract
J. Biol. Chem., September 27, 2002; 277(40): 37536 - 37541.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
E. Fossale, V. C. Wheeler, V. Vrbanac, L.-A. Lebel, A. Teed, J. S. Mysore, J. F. Gusella, M. E. MacDonald, and F. Persichetti
Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice
Hum. Mol. Genet., September 15, 2002; 11(19): 2233 - 2241.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
H. Kita, J. Carmichael, J. Swartz, S. Muro, A. Wyttenbach, K. Matsubara, D. C. Rubinsztein, and K. Kato
Modulation of polyglutamine-induced cell death by genes identified by expression profiling
Hum. Mol. Genet., September 15, 2002; 11(19): 2279 - 2287.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
R. Luthi-Carter, S. A. Hanson, A. D. Strand, D. A. Bergstrom, W. Chun, N. L. Peters, A. M. Woods, E. Y. Chan, C. Kooperberg, D. Krainc, et al.
Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain
Hum. Mol. Genet., August 15, 2002; 11(17): 1911 - 1926.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
R. Luthi-Carter, A. D. Strand, S. A. Hanson, C. Kooperberg, G. Schilling, A. R. La Spada, D. E. Merry, A. B. Young, C. A. Ross, D. R. Borchelt, et al.
Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects
Hum. Mol. Genet., August 15, 2002; 11(17): 1927 - 1937.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
A. P. Lieberman, G. Harmison, A. D. Strand, J. M. Olson, and K. H. Fischbeck
Altered transcriptional regulation in cells expressing the expanded polyglutamine androgen receptor
Hum. Mol. Genet., August 15, 2002; 11(17): 1967 - 1976.
[Abstract] [Full Text] [PDF]


Home page
Proc. Natl. Acad. Sci. USAHome page
Y. Chai, J. Shao, V. M. Miller, A. Williams, and H. L. Paulson
Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis
PNAS, July 9, 2002; 99(14): 9310 - 9315.
[Abstract] [Full Text] [PDF]


Home page
BrainHome page
J. Takahashi, H. Fujigasaki, C. Zander, K. H. El Hachimi, G. Stevanin, A. Durr, A.-S. Lebre, G. Yvert, Y. Trottier, H. d. The, et al.
Two populations of neuronal intranuclear inclusions in SCA7 differ in size and promyelocytic leukaemia protein content
Brain, July 1, 2002; 125(7): 1534 - 1543.
[Abstract] [Full Text] [PDF]


Home page
J. Neurosci.Home page
G. A. Garden, R. T. Libby, Y.-H. Fu, Y. Kinoshita, J. Huang, D. E. Possin, A. C. Smith, R. A. Martinez, G. C. Fine, S. K. Grote, et al.
Polyglutamine-Expanded Ataxin-7 Promotes Non-Cell-Autonomous Purkinje Cell Degeneration and Displays Proteolytic Cleavage in Ataxic Transgenic Mice
J. Neurosci., June 15, 2002; 22(12): 4897 - 4905.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
Z.-X. Yu, S.-H. Li, H.-P. Nguyen, and X.-J. Li
Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice
Hum. Mol. Genet., April 15, 2002; 11(8): 905 - 914.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
C. K. Bailey, I. F. M. Andriola, H. H. Kampinga, and D. E. Merry
Molecular chaperones enhance the degradation of expanded polyglutamine repeat androgen receptor in a cellular model of spinal and bulbar muscular atrophy
Hum. Mol. Genet., March 1, 2002; 11(5): 515 - 523.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
W. J. Welch and M. I. Diamond
Glucocorticoid modulation of androgen receptor nuclear aggregation and cellular toxicity is associated with distinct forms of soluble expanded polyglutamine protein
Hum. Mol. Genet., December 1, 2001; 10(26): 3063 - 3074.
[Abstract] [Full Text] [PDF]


Home page
Am. J. Pathol.Home page
M. Yamada, T. Sato, T. Shimohata, S. Hayashi, S. Igarashi, S. Tsuji, and H. Takahashi
Interaction between Neuronal Intranuclear Inclusions and Promyelocytic Leukemia Protein Nuclear and Coiled Bodies in CAG Repeat Diseases
Am. J. Pathol., November 1, 2001; 159(5): 1785 - 1795.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
K. Sathasivam, B. Woodman, A. Mahal, F. Bertaux, E. E. Wanker, D. T. Shima, and G. P. Bates
Centrosome disorganization in fibroblast cultures derived from R6/2 Huntington's disease (HD) transgenic mice and HD patients
Hum. Mol. Genet., October 1, 2001; 10(21): 2425 - 2435.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
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Hum. Mol. Genet., August 1, 2001; 10(16): 1679 - 1692.
[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
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Hum. Mol. Genet., August 1, 2001; 10(17): 1829 - 1845.
[Abstract] [Full Text] [PDF]


Home page
J. Neurosci.Home page
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[Abstract] [Full Text] [PDF]


Home page
Hum Mol GenetHome page
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Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death
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[Abstract] [Full Text] [PDF]


Home page
ScienceHome page
F. C. Nucifora Jr., M. Sasaki, M. F. Peters, H. Huang, J. K. Cooper, M. Yamada, H. Takahashi, S. Tsuji, J. Troncoso, V. L. Dawson, et al.
Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity
Science, March 23, 2001; 291(5512): 2423 - 2428.
[Abstract] [Full Text]


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Proc. Natl. Acad. Sci. USAHome page
S. Holbert, I. Denghien, T. Kiechle, A. Rosenblatt, C. Wellington, M. R. Hayden, R. L. Margolis, C. A. Ross, J. Dausset, R. J. Ferrante, et al.
The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: Neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis
PNAS, January 24, 2001; (2001) 41566798.
[Abstract] [Full Text]


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Proc. Natl. Acad. Sci. USAHome page
M. F. Beal and P. Hantraye
Novel therapies in the search for a cure for Huntington's disease
PNAS, January 2, 2001; 98(1): 3 - 4.
[Full Text] [PDF]


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Hum Mol GenetHome page
A. Abel, J. Walcott, J. Woods, J. Duda, and D. E. Merry
Expression of expanded repeat androgen receptor produces neurologic disease in transgenic mice
Hum. Mol. Genet., January 1, 2001; 10(2): 107 - 116.
[Abstract] [Full Text] [PDF]


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Hum Mol GenetHome page
F. Trettel, D. Rigamonti, P. Hilditch-Maguire, V. C. Wheeler, A. H. Sharp, F. Persichetti, E. Cattaneo, and M. E. MacDonald
Dominant phenotypes produced by the HD mutation in STHdhQ111 striatal cells
Hum. Mol. Genet., November 1, 2000; 9(19): 2799 - 2809.
[Abstract] [Full Text] [PDF]


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J. Biol. Chem.Home page
M. F. Peters and C. A. Ross
Isolation of a 40-kDa Huntingtin-associated Protein
J. Biol. Chem., January 26, 2001; 276(5): 3188 - 3194.
[Abstract] [Full Text] [PDF]


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J. Biol. Chem.Home page
Y. Chai, L. Wu, J. D. Griffin, and H. L. Paulson
The Role of Protein Composition in Specifying Nuclear Inclusion Formation in Polyglutamine Disease
J. Biol. Chem., November 21, 2001; 276(48): 44889 - 44897.
[Abstract] [Full Text] [PDF]


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Proc. Natl. Acad. Sci. USAHome page
S. Holbert, I. Denghien, T. Kiechle, A. Rosenblatt, C. Wellington, M. R. Hayden, R. L. Margolis, C. A. Ross, J. Dausset, R. J. Ferrante, et al.
The Gln-Ala repeat transcriptional activator CA150 interacts with huntingtin: Neuropathologic and genetic evidence for a role in Huntington's disease pathogenesis
PNAS, February 13, 2001; 98(4): 1811 - 1816.
[Abstract] [Full Text] [PDF]


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Proc. Natl. Acad. Sci. USAHome page
R. E. Hughes, R. S. Lo, C. Davis, A. D. Strand, C. L. Neal, J. M. Olson, and S. Fields
Altered transcription in yeast expressing expanded polyglutamine
PNAS, November 6, 2001; 98(23): 13201 - 13206.
[Abstract] [Full Text] [PDF]


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Proc. Natl. Acad. Sci. USAHome page
A. McCampbell, A. A. Taye, L. Whitty, E. Penney, J. S. Steffan, and K. H. Fischbeck
Histone deacetylase inhibitors reduce polyglutamine toxicity
PNAS, December 18, 2001; 98(26): 15179 - 15184.
[Abstract] [Full Text] [PDF]



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