SEARCH

SEARCH BY CITATION

References

  • Boutell J. M., Thomas P., Neal J. W., Weston V. J., Duce J., Harper P. S. and Jones A. L. (1999) Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum. Mol. Genet. 8, 16471655.
  • Busch A., Engemann S., Lurz R., Okazawa H., Lehrach H. and Wanker E. E. (2003) Mutant huntingtin promotes the fibrillogenesis of wild type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease. J. Biol. Chem. 278, 4145241461.
  • Chai Y., Shao J., Miller V. M., Williams A. and Paulson H. L. (2002) Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis. Proc. Natl Acad. Sci. USA 99, 93109315.
  • Cummings C. J. and Zoghbi H. Y. (2000) Fourteen and counting: unraveling trinucleotide repeat diseases. Hum. Mol. Genet. 9, 909916.
  • Davies S. W., Turmaine M., Cozens B. A., DiFiglia M., Sharp A. H., Ross C. A., Scherzinger E., Wanker E. E., Mangiarini L. and Bates G. P. (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537548.
  • Diamond M. I., Robinson M. R. and Yamamoto K. R. (2000) Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor. Proc. Natl Acad. Sci. USA 97, 657661.
  • 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, 19901993.
  • Dragatsis I., Levine M. S. and Zeitlin S. (2000) Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat. Genet. 26, 300306.
  • Dunah A. W., Jeong H., Griffin A., Kim Y. M., Standaert D. G., Hersch S. M., Mouradian M. M., Young A. B., Tanese N. and Krainc D. (2002) Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 296, 22382243.
  • Duyckaerts C., Durr A., Cancel G. and Brice A. (1999) Nuclear inclusions in spinocerebellar ataxia type 1. Acta Neuropathol (Berl) 97, 201207.
  • Dyer R. B. and McMurray C. T. (2001) Mutant protein in Huntington disease is resistant to proteolysis in affected brain. Nat. Genet. 29, 270278.
  • Fadok V. A., Laszlo D. J., Noble P. W., Weinstein L., Riches D. W. and Henson P. M. (1993) Particle digestibility is required for induction of the phosphatidylserine recognition mechanism used by murine macrophages to phagocytose apoptotic cells. J. Immunol. 151, 42744285.
  • Fadok V. A., Voelker D. R., Campbell P. A., Cohen J. J., Bratton D. L. and Henson P. M. (1992) Exposure of phosphatidylserine on the surface of apoptotic lymphocytes triggers specific recognition and removal by macrophages. J. Immunol. 148, 22072216.
  • Gusella J. F. and MacDonald M. E. (2000) Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat. Rev. Neurosci. 1, 109115.
  • Hackam A. S., Singaraja R., Zhang T., Gan L. and Hayden M. R. (1999) In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease. Hum. Mol. Genet. 8, 2533.
  • Heiser V., Scherzinger E., Boeddrich A., Nordhoff E., LurZ R., Schugardt N., Lehrach H. and Wanker E. E. (2000) Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: implications for Huntington's disease therapy. Proc. Natl Acad. Sci. U S A 97, 67396744.
  • Hodgson J. G., Agopyan N., Gutekunst C.-A. et al. (1999) A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity and selective striatal neurodegeneration. Neuron 23, 181192.
  • Hoshino M., Tagawa K., Okuda T., Murata M., Oyanagi K., Arai N., Mizutani T., Kanazawa I., Wanker E. E. and Okazawa H. (2003) Histone deacetylase activity is retained in primary neurons expressing mutant huntingtin protein. J. Neurochem. 87, 257267.
  • Hsiao P. W., Lin D. L., Nakao R. and Chang C. (1999) The linkage of Kennedy's neuron disease to ARA24, the first identified androgen receptor polyglutamine region-associated coactivator. J. Biol. Chem. 274, 2022920234.
  • Irvine R. A., Ma H. Yu M. C., Ross R. K., Stallcup M. R. and Coetzee G. A. (2000) Inhibition of p160-mediated coactivation with increasing androgen receptor polyglutamine length. Hum. Mol. Genet. 9, 267274.
  • Kazantsev A., Preisinger E., Dranovsky A., Goldgaber D. and Housman D. (1999) Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc. Natl Acad. Sci. USA 96, 1140411409.
  • Kazemi-Esfarjani P. and Benzer S. (2000) Genetic suppression of polyglutamine toxicity in Drosophila. Science 287, 18371840.
  • Kegel K. B., Meloni A. R., Yi Y. et al. (2002) Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J. Biol. Chem. 277, 74667476.
  • Kim S., Nollen E. A., Kitagawa K., Bindokas V. P. and Morimoto R. I. (2002) Polyglutamine protein aggregates are dynamic. Nat. Cell Biol. 4, 826831.
  • 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, 4153.
  • Kuemmerle S., Gutekunst C. A., Klein A. M., Li X. J., Li S. H., Beal M. F., Hersch S. M. and Ferrante R. J. (1999) Huntington aggregates may not predict neuronal death in Huntington's disease. Anal. Neurol. 46, 842849.
  • Lescure A., Lutz Y., Eberhard D., Jacq X., Krol A., Grummt I., Davidson I., Chambon P. and Tora L. (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, 11661175.
  • Li H., Li S. H., Johnston H., Shelbourne P. F. and Li X. J. (2000) Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity. Nat. Genet. 25, 385389.
  • Mantamadiotis T., Lemberger T., Bleckmann S. C. et al. (2002) Disruption of CREB function in brain leads to neurodegeneration. Nat. Genet. 31, 4754.
  • Martin-Aparicio E., Avila J. and Lucas J. J. (2002) Nuclear localization of N-terminal mutant huntingtin is cell cycle dependent. Eur. J. Neurosci. 16, 355359.
  • McCampbell A., Taye A. A., Whitty L., Penney E., Steffan J. S. and Fischbeck K. H. (2001) Histone deacetylase inhibitors reduce polyglutamine toxicity. Proc. Natl Acad. Sci. USA 98, 1517915184.
  • McCampbell A., Taylor J. P., Taye A. A. et al. (2000) CREB-binding protein sequestration by expanded polyglutamine. Hum. Mol. Genet. 9, 21972202.
  • Nucifora F. C. Jr, Sasaki M., Peters M. F. et al. (2001) Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 291, 24232428.
  • Okazawa H., Rich T., Chang A. et al. (2002) Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death. Neuron 34, 701713.
  • Paulson H. L., Perez M. K., Trottier Y., Trojanowski J. Q., Subramony S. H., Das S. S., Vig P., Mandel J. L., Fischbeck K. H. and Pittman R. N. (1997) Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333344.
  • 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, 14571470.
  • Perutz M. F. and Windle A. H. (2001) Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats. Nature 412, 143144.
  • Rich T., Assier E., Skepper J., Segard H. B., Allen R. L., Charron D. and Trowsdale J. (1999) Disassembly of nuclear inclusions in the dividing cell – a novel insight into neurodegeneration. Hum. Mol. Genet. 8, 24512459.
  • Ross C. A. (2002) Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders. Neuron 35, 819822.
  • Sanchez I., Mahlke C. and Yuan J. (2003) Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 421, 373379.
  • 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, 5566.
  • Scherzinger E., Lurz R., Turmaine M., Mangiarini L., Hollenbach B., Hasenbank R., Bates G. P., Davies S. W., Lehrach H. and Wanker E. E. (1997) Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549558.
  • Shimohata T., Nakajima T., Yamada M. et al. (2000) Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet. 26, 2936.
  • Simeoni S., Mancini M. A., Stenoien D. L., Marcelli M., Weigel N. L., Zanisi M., Martini L. and Poletti A. (2000) Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract. Hum. Mol. Genet. 9, 133144.
  • Sittler A., Lurz R., Lueder G., Priller J., Lehrach H., Hayer-Hartl M. K., Hartl F. U. and Wanker E. E. (2001) Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease. Hum. Mol. Genet. 10, 13071315.
  • Sittler A., Walter S., Wedemeyer N., Hasenbank R., Scherzinger E., Eickhoff H., Bates G. P., Lehrach H. and Wanker E. E. (1998) SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol. Cell 2, 427436.
  • Steffan J. S., Bodai L., Pallos J. et al. (2001) Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 413, 739743.
  • Steffan J. S., Kazantsev A., Spasic-Boskovic O., Greenwald M., Zhu Y. Z., Gohler H., Wanker E. E., Bates G. P., Housman D. E. and Thompson L. M. (2000) The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc. Natl Acad. Sci. USA 97, 67636768.
  • 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, 731741.
  • Stenoien D. L., Mielke M. and Mancini M. A. (2002) Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components. Nat. Cell Biol. 4, 806810.
  • Suhr S. T., Senut M. C., Whitelegge J. P., Faull K. F., Cuizon D. B. and Gage F. H. (2001) Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression. J. Cell Biol. 153, 283294.
  • Turmaine M., Raza A., Mahal A., Mangiarini L., Bates G. P. and Davies S. W. (2000) Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proc. Natl Acad. Sci. USA 97, 80938097.
  • Waelter S., Boeddrich A., Lurz R., Scherzinger E., Lueder G., Lehrach H. and Wanker E. E. (2001) Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Mol. Biol. Cell 12, 13931407.
  • Waragai M., Lammers C. H., Takeuchi S., Imafuku I., Udagawa Y., Kanazawa I., Kawabata M., Mouradian M. M. and Okazawa H. (1999) PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival. Hum. Mol. Genet. 8, 977987.
  • Warrick J. M., Chan H. Y., Gray-Board G. L., Chai Y., Paulson H. L. and Bonini N. M. (1999) Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet. 23, 425428.
  • Wood J. D., Nucifora F. C. Jr, Duan K., Zhang C., Wang J., Kim Y., Schilling G., Sacchi N., Liu J. M. and Ross C. A. (2000) Atrophin-1, the dentato-rubral and pallido-luysian atrophy gene product, interacts with ETO/MTG8 in the nuclear matrix and represses transcription. J. Cell Biol. 150, 939948.
  • Yamamoto A., Lucas J. J. and Hen R. (2000) Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101, 5766.
  • Yvert G., Lindenberg K. S., Devys D., Helmlinger D., Landwehrmeyer G. B. and Mandel J. L. (2001) SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types. Hum. Mol. Genet. 10, 16791692.
  • Zuccato C., Tartari M., Crotti A., Goffredo D., Valenza M., Conti L., Cataudella T., Leavitt B. R., . Hayden M. R., Timmusk T., et al. (2003) Huntingtin interacts with. REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat. Genet. 35, 7683.