SEARCH

SEARCH BY CITATION

References

  • Arrasate M., Mitra S., Schweitzer E. S., Segal M. R. and Finkbeiner S. (2004) Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431, 805810.
  • Bence N. F., Sampat R. M. and Kopito R. R. (2001) Impairment of ubiquitin–proteasome system by protein aggregation. Science 292, 15521555.
  • Bennett E. J., Bence N. F., Jayakumar R. and Kopito R. R. (2005) Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. Mol. Cell 17, 351365.
  • Bowman A. B., Yoo S. Y., Dantuma N. P. and Zoghbi H. Y. (2005) Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin–proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation. Hum. Mol. Genet. 14, 679691.
  • Brenner S. (1974) The genetics of Caenorhabditis elegans. Genetics 77, 7194.
  • Bucciantini M., Dobson C. M. and Stefani M. (2002) Inherent toxicity of aggregates implies a common mechanism of protein misfolding diseases. Nature 416, 507511.
  • Burnett B., Li F. and Pittman R. N. (2003) The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity. Hum. Mol. Genet. 12, 31953205.
  • Chalfie M. and White J. (1988) The nervous system, in The Nematode Caenorhabditis Elegans (WoodW. B., ed.), pp. 337391. Cold Spring Harbor Laboratory Press, Cold Spring Harbor.
  • Ferrante R. J., Kowall N. W. and Richardson E. P. Jr (1991) Proliferative and degenerative changes in striatal spiny neurons in Huntington's disease: a combined study using the section-Golgi method and calbindin D28k immunocytochemistry. J. Neurosci. 11, 38773887.
  • Gatchel J. R. and Zoghbi H. Y. (2005) Diseases of unstable repeat expansion: mechanisms and common principles. Nat. Rev. Genet. 6, 743755.
  • Gunawardena S., Her L. S., Brusch R. G., Laymon R. A., Niesman I. R., Gold B. G., Sintasath L., Binini N. M. and Goldstein L. S. B. (2003) Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila. Neuron 40, 2540.
  • Harry G. J. (1992) Acrylamide-induced alterations in axonal transport. Biochemical and autoradiographic studies. Mol. Neurobiol. 6, 203216.
  • Holmberg C. I., Staniszewski K. E., Mensah K. N., Matouschek A. and Morimoto R. I. (2004) Inefficient degradation of truncated polyglutamine proteins by the proteasome. EMBO J. 23, 43074318.
  • Jana N. R. and Nukina N. (2004) Misfolding promotes the ubiquitination of polyglutamine-expanded ataxin-3, the defective gene product in SCA3/MJD. Neurotox. Res. 6, 523533.
  • Jana N. R., Zemskov E. A., Wang G. and Nukina N. (2001) Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through through mitochondrial cytochrome c release. Hum. Mol. Genet. 10, 10491059.
  • Kawaguchi Y., Okamoto T., Taniwaki M. et al. (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet. 8, 221228.
  • Kraemer B. C., Zhang B., Leverenz J. B., Thomas J. H., Trojanowski J. Q. and Schellenberg G. D. (2003) Neurodegeneration and defective neurotransmission in a Caenorhabditis elegans model of tauopathy. Proc. Natl Acad. Sci. USA 100, 99809985.
  • Lundquist E. A., Reddien P. W., Hartwieg E., HorvitZ. H. R. and Bargmann C. I. (2001) Three C. elegans Rac proteins and several alternative Rac regulators control axon guidance, cell migration and apoptotic cell phagocytosis. Development 128, 44754488.
  • Maciel P., Gasper C., DeStefano A. L., Silveira I., Coutinho P., Radvany J., Dawson D. M., Sudarsky L., Guimeres J., Loureiro J. E. L. et al. (1995) Correlation between CAG repeat length and clinical features in Machado-Joseph disease. Am. J. Hum. Genet. 57, 5461.
  • Nagaoka U., Kim K., Jana N. R., Doi H., Maruyama M., Mitsui K., Oyama F. and Nukina N. (2004) Increased expression of p62 in expanded polyglutamine-expressing cells and its association with polyglutamine inclusions. J. Neurochem. 91, 5768.
  • Nicniocaill B., Haraldsson B., Hansson O., O'Connor W. T. and Brundin P. (2001) Altered striatal amino acid neurotransmitter release monitored using microdialysis in R6/1 Huntington transgenic mice. Eur. J. Neurosci. 13, 206210.
  • Nonet M. L., Grundahl K., Meyer B. and Rand J. B. (1993) Synaptic function is impaired but not eliminated in C. elegans mutants lacking synaptotagmin. Cell 73, 12911305.
  • 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.
  • Parker J. A., Connolly J. B., Wellington C., Hayden M., Dausset J. and Neri C. (2001) Expanded polyglutamines in Caenorhabditis elegans cause axonal abnormalities and severe dysfunction of PLM mechanosensory neurons without cell death. Proc. Natl Acad. Sci. USA 98, 13 31813 323.
  • Paulson H. L. (1999) Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the misfold. Am. J. Hum. Genet. 64, 339345.
  • Piccioni F., Pinton P., Simeoni S., Pozzi P., Fascio U., Vismara G., Martini L., Rizzuto R. and Poletti A. (2002) Androgen receptor with elongated polyglutamine tract forms aggregates that alter axonal trafficking and mitochondrial distribution in motor neuronal processes. FASEB J. 16, 14181420.
  • Roediger B. and Armati P. J. (2003) Oxidative stress induces axonal beading in cultured human brain tissue. Neurobiol. Dis. 13, 222229.
  • Rosenberg R. N. (1992) Machado-Joseph disease: a autosomal dominant motor system degeneration. Mov. Disord. 7, 193203.
  • Ross C. A. (2002) Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders. Neuron 35, 819822.
  • Szebenyi G., Morfini G. A., Babcock A. et al. (2003) Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport. Neuron 40, 4152.
  • Tanaka Y., Engelender S., Igarashi S., Rao R. K., Wanner T., Tanzi R. E., Sawa A. L., Dawson V., Dawson T. M. and Ross C. A. (2001) Inducible expression of mutant alpha-synuclein decreases proteasome activity and increases sensitivity to mitochondria-dependent apoptosis. Hum. Mol. Genet. 10, 919926.
  • Venkatraman P., Wetzel R., Tanaka M., Nukina N. and Goldberg A. L. (2004) Eukaryotic proteasomes cannot digest polyglutamine sequences and release them during degradation of polyglutamine-containing proteins. Mol. Cell 14, 95104.
  • Voisine C. and Hart A. C. (2004) Caenorhabditis elegans as a model system for triplet repeat diseases. Methods Mol. Biol. 277, 141160.
  • Wang G. H., Mitsui K., Kotliarova S., Yamashita A., Nagao Y., Tokuhiro S., Iwatsubo T., Kanazawa I. and Nukina N. (1999) Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells. Neuroreport 10, 24352438.
  • Wang G., Sawai N., Kotliarova S., Kanazawa I. and Nukina N. (2000) Ataxin-3, the MJD1 gene product, interacts with the two human homologs of yeast DNA repair protein RAD23, HHR23A and HHR23B. Hum. Mol. Genet. 9, 17951803.
  • White J. G., Southgate E., Thomson J. N. and Brenner S. (1986) The structure of the nervous system of the nematode Caenorhabditis elegans. Philos. Trans. R. Soc. Lond. 314, 1340.
  • Zemskov E. A. and Nukina N. (2003) Impaired degradation of PKCalpha by proteasome in a cellular model of Huntington's disease. Neuroreport 14, 14351438.