Human Molecular Genetics, Vol 8, 731-741, Copyright © 1999 by Oxford University Press
DL Stenoien, CJ Cummings, HP Adams, MG Mancini, K Patel, GN DeMartino, M Marcelli, NL Weigel and MA Mancini
Spinal bulbar muscular atrophy is a neurodegenerative disorder caused by a
polyglutamine expansion in the androgen receptor (AR). We show in
transiently transfected HeLa cells that an AR containing 48 glutamines
(ARQ48) accumulates in a hormone-dependent manner in both cytoplasmic and
nuclear aggregates. Electron microscopy reveals both types of aggregates to
have a similar ultrastructure. ARQ48 aggregates sequester mitochondria and
steroid receptor coactivator 1 and stain positively for NEDD8, Hsp70, Hsp90
and HDJ-2/HSDJ. Co-expression of HDJ-2/HSDJ significantly represses
aggregate formation. ARQ48 aggregates also label with antibodies
recognizing the PA700 proteasome caps but not 20S core particles. These
results suggest that ARQ48 accumulates due to protein misfolding and a
breakdown in proteolytic processing. Furthermore, the homeostatic
disturbances associated with aggregate formation may affect normal cell
function.
ARTICLES
Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone
Department of Cell Biology, Baylor College of Medicine and VA Medical Center, One Baylor Plaza, Houston, TX 77030, USA.
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A. Wyttenbach, J. Swartz, H. Kita, T. Thykjaer, J. Carmichael, J. Bradley, R. Brown, M. Maxwell, A. Schapira, T. F. Orntoft, et al. Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease Hum. Mol. Genet., August 1, 2001; 10(17): 1829 - 1845. [Abstract] [Full Text] [PDF]
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Y. Giguere, E. Dewailly, J. Brisson, P. Ayotte, N. Laflamme, A. Demers, V.-I. Forest, S. Dodin, J. Robert, and F. Rousseau Short Polyglutamine Tracts in the Androgen Receptor Are Protective against Breast Cancer in the General Population Cancer Res., August 1, 2001; 61(15): 5869 - 5874. [Abstract] [Full Text] [PDF]
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A. B. Meriin, K. Mabuchi, V. L. Gabai, J. A. Yaglom, A. Kazantsev, and M. Y. Sherman Intracellular Aggregation of Polypeptides with Expanded Polyglutamine Domain Is Stimulated by Stress-Activated Kinase Mekk1 J. Cell Biol., May 14, 2001; 153(4): 851 - 864. [Abstract] [Full Text] [PDF]
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H. Adachi, A. Kume, M. Li, Y. Nakagomi, H. Niwa, J. Do, C. Sang, Y. Kobayashi, M. Doyu, and G. Sobue Transgenic mice with an expanded CAG repeat controlled by the human AR promoter show polyglutamine nuclear inclusions and neuronal dysfunction without neuronal cell death Hum. Mol. Genet., May 1, 2001; 10(10): 1039 - 1048. [Abstract] [Full Text] [PDF]
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N. R. Jana, E. A. Zemskov, G.-h. Wang, and N. Nukina Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release Hum. Mol. Genet., May 1, 2001; 10(10): 1049 - 1059. [Abstract] [Full Text] [PDF]
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A.-S. Lebre, L. Jamot, J. Takahashi, N. Spassky, C. Leprince, N. Ravise, C. Zander, H. Fujigasaki, P. Kussel-Andermann, C. Duyckaerts, et al. Ataxin-7 interacts with a Cbl-associated protein that it recruits into neuronal intranuclear inclusions Hum. Mol. Genet., May 1, 2001; 10(11): 1201 - 1213. [Abstract] [Full Text] [PDF]
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S. Waelter, A. Boeddrich, R. Lurz, E. Scherzinger, G. Lueder, H. Lehrach, and E. E. Wanker Accumulation of Mutant Huntingtin Fragments in Aggresome-like Inclusion Bodies as a Result of Insufficient Protein Degradation Mol. Biol. Cell, May 1, 2001; 12(5): 1393 - 1407. [Abstract] [Full Text]
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H.Y. E. Chan, J. M. Warrick, G. L. Gray-Board, H. L. Paulson, and N. M. Bonini Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila Hum. Mol. Genet., November 1, 2000; 9(19): 2811 - 2820. [Abstract] [Full Text] [PDF]
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G. Yvert, K. S. Lindenberg, S. Picaud, G. B. Landwehrmeyer, J.-A. Sahel, and J.-L. Mandel Expanded polyglutamines induce neurodegeneration and trans-neuronal alterations in cerebellum and retina of SCA7 transgenic mice Hum. Mol. Genet., October 1, 2000; 9(17): 2491 - 2506. [Abstract] [Full Text] [PDF]
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A. McCampbell, J. P. Taylor, A. A. Taye, J. Robitschek, M. Li, J. Walcott, D. Merry, Y. Chai, H. Paulson, G. Sobue, et al. CREB-binding protein sequestration by expanded polyglutamine Hum. Mol. Genet., September 1, 2000; 9(14): 2197 - 2202. [Abstract] [Full Text] [PDF]
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A. Calado, F. M.S. Tome, B. Brais, G.A. Rouleau, U. Kuhn, E. Wahle, and M. Carmo-Fonseca Nuclear inclusions in oculopharyngeal muscular dystrophy consist of poly(A) binding protein 2 aggregates which sequester poly(A) RNA Hum. Mol. Genet., September 1, 2000; 9(15): 2321 - 2328. [Abstract] [Full Text] [PDF]
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N. R. Jana, M. Tanaka, G.-h. Wang, and N. Nukina Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity Hum. Mol. Genet., August 12, 2000; 9(13): 2009 - 2018. [Abstract] [Full Text] [PDF]
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M. Becker, E. Martin, J. Schneikert, H. F. Krug, and A. C.B. Cato Cytoplasmic Localization and the Choice of Ligand Determine Aggregate Formation by Androgen Receptor with Amplified Polyglutamine Stretch J. Cell Biol., April 17, 2000; 149(2): 255 - 262. [Abstract] [Full Text] [PDF]
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C. J. Cummings and H. Y. Zoghbi Fourteen and counting: unraveling trinucleotide repeat diseases Hum. Mol. Genet., April 1, 2000; 9(6): 909 - 916. [Abstract] [Full Text] [PDF]
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H.N. Lim, H. Chen, S. McBride, A.M. Dunning, R.M. Nixon, I.A. Hughes, and J.R. Hawkins Longer polyglutamine tracts in the androgen receptor are associated with moderate to severe undermasculinized genitalia in XY males Hum. Mol. Genet., March 22, 2000; 9(5): 829 - 834. [Abstract] [Full Text] [PDF]
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Y. Kobayashi, A. Kume, M. Li, M. Doyu, M. Hata, K. Ohtsuka, and G. Sobue Chaperones Hsp70 and Hsp40 Suppress Aggregate Formation and Apoptosis in Cultured Neuronal Cells Expressing Truncated Androgen Receptor Protein with Expanded Polyglutamine Tract J. Biol. Chem., March 17, 2000; 275(12): 8772 - 8778. [Abstract] [Full Text] [PDF]
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A. Wyttenbach, J. Carmichael, J. Swartz, R. A. Furlong, Y. Narain, J. Rankin, and D. C. Rubinsztein Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease PNAS, March 14, 2000; 97(6): 2898 - 2903. [Abstract] [Full Text] [PDF]
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S. Krobitsch and S. Lindquist Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins PNAS, February 15, 2000; 97(4): 1589 - 1594. [Abstract] [Full Text] [PDF]
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R. A. Irvine, H. Ma, M. C. Yu, R. K. Ross, M. R. Stallcup, and G. A. Coetzee Inhibition of p160-mediated coactivation with increasing androgen receptor polyglutamine length Hum. Mol. Genet., January 22, 2000; 9(2): 267 - 274. [Abstract] [Full Text] [PDF]
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Z Dastoor and J Dreyer Nuclear translocation and aggregate formation of heat shock cognate protein 70 (Hsc70) in oxidative stress and apoptosis J. Cell Sci., January 8, 2000; 113(16): 2845 - 2854. [Abstract] [PDF]
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S. Simeoni, M. A. Mancini, D. L. Stenoien, M. Marcelli, N. L. Weigel, M. Zanisi, L. Martini, and A. Poletti Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract Hum. Mol. Genet., January 1, 2000; 9(1): 133 - 144. [Abstract] [Full Text] [PDF]
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M. D. Kaytor and S. T. Warren Aberrant Protein Deposition and Neurological Disease J. Biol. Chem., December 31, 1999; 274(53): 37507 - 37510. [Full Text] [PDF]
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L. V. Nazareth, D. L. Stenoien, W. E. Bingman III, A. J. James, C. Wu, Y. Zhang, D. P. Edwards, M. Mancini, M. Marcelli, D. J. Lamb, et al. A C619Y Mutation in the Human Androgen Receptor Causes Inactivation and Mislocalization of the Receptor with Concomitant Sequestration of SRC-1 (Steroid Receptor Coactivator 1) Mol. Endocrinol., December 1, 1999; 13(12): 2065 - 2075. [Abstract] [Full Text]
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Y. Chai, S. L. Koppenhafer, N. M. Bonini, and H. L. Paulson Analysis of the Role of Heat Shock Protein (Hsp) Molecular Chaperones in Polyglutamine Disease J. Neurosci., December 1, 1999; 19(23): 10338 - 10347. [Abstract] [Full Text] [PDF]
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K. Terada and M. Mori Human DnaJ Homologs dj2 and dj3, and bag-1 Are Positive Cochaperones of hsc70 J. Biol. Chem., August 4, 2000; 275(32): 24728 - 24734. [Abstract] [Full Text] [PDF]
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G. A. Shinder, M.-C. Lacourse, S. Minotti, and H. D. Durham Mutant Cu/Zn-Superoxide Dismutase Proteins Have Altered Solubility and Interact with Heat Shock/Stress Proteins in Models of Amyotrophic Lateral Sclerosis J. Biol. Chem., April 13, 2001; 276(16): 12791 - 12796. [Abstract] [Full Text] [PDF]
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S. H. Satyal, E. Schmidt, K. Kitagawa, N. Sondheimer, S. Lindquist, J. M. Kramer, and R. I. Morimoto Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans PNAS, May 23, 2000; 97(11): 5750 - 5755. [Abstract] [Full Text] [PDF]
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