You have full text access to this OnlineOpen article
Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein
Article first published online: 16 APR 2004
DOI: 10.1111/j.1471-4159.2004.02372.x
Additional Information
How to Cite
Tagawa, K., Hoshino, M., Okuda, T., Ueda, H., Hayashi, H., Engemann, S., Okado, H., Ichikawa, M., Wanker, E. E. and Okazawa, H. (2004), Distinct aggregation and cell death patterns among different types of primary neurons induced by mutant huntingtin protein. Journal of Neurochemistry, 89: 974–987. doi: 10.1111/j.1471-4159.2004.02372.x
Publication History
- Issue published online: 16 APR 2004
- Article first published online: 16 APR 2004
- Resubmitted manuscript received December 27, 2003; accepted January 1, 2004.
References
- , , , , , and (1999) Aberrant interactions of transcriptional repressor proteins with the Huntington's disease gene product, huntingtin. Hum. Mol. Genet. 8, 1647–1655.
- , , , , and (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, 41452–41461.
- , , , and (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, 9310–9315.
- and (2000) Fourteen and counting: unraveling trinucleotide repeat diseases. Hum. Mol. Genet. 9, 909–916.
- , , , , , , , , and (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537–548.
- , and (2000) Regulation of expanded polyglutamine protein aggregation and nuclear localization by the glucocorticoid receptor. Proc. Natl Acad. Sci. USA 97, 657–661.
- , , , , , and (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990–1993.
- , and (2000) Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat. Genet. 26, 300–306.
- , , , , , , , , and (2002) Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 296, 2238–2243.
- , , and (1999) Nuclear inclusions in spinocerebellar ataxia type 1. Acta Neuropathol (Berl) 97, 201–207.
- and (2001) Mutant protein in Huntington disease is resistant to proteolysis in affected brain. Nat. Genet. 29, 270–278.
- , , , , and (1993) Particle digestibility is required for induction of the phosphatidylserine recognition mechanism used by murine macrophages to phagocytose apoptotic cells. J. Immunol. 151, 4274–4285.
- , , , , and (1992) Exposure of phosphatidylserine on the surface of apoptotic lymphocytes triggers specific recognition and removal by macrophages. J. Immunol. 148, 2207–2216.
- and (2000) Molecular genetics: unmasking polyglutamine triggers in neurodegenerative disease. Nat. Rev. Neurosci. 1, 109–115.
- , , , and (1999) In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease. Hum. Mol. Genet. 8, 25–33.
- , , , , , , and (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, 6739–6744.
- , , et al. (1999) A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity and selective striatal neurodegeneration. Neuron 23, 181–192.
- , , , , , , , , and (2003) Histone deacetylase activity is retained in primary neurons expressing mutant huntingtin protein. J. Neurochem. 87, 257–267.
- , , and (1999) The linkage of Kennedy's neuron disease to ARA24, the first identified androgen receptor polyglutamine region-associated coactivator. J. Biol. Chem. 274, 20229–20234.
- , , , and (2000) Inhibition of p160-mediated coactivation with increasing androgen receptor polyglutamine length. Hum. Mol. Genet. 9, 267–274.
- , , , and (1999) Insoluble detergent-resistant aggregates form between pathological and nonpathological lengths of polyglutamine in mammalian cells. Proc. Natl Acad. Sci. USA 96, 11404–11409.
- and (2000) Genetic suppression of polyglutamine toxicity in Drosophila. Science 287, 1837–1840.
- , , 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, 7466–7476.
- , , , and (2002) Polyglutamine protein aggregates are dynamic. Nat. Cell Biol. 4, 826–831.
- , , , , , , and (1998) Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95, 41–53.
- , , , , , , and (1999) Huntington aggregates may not predict neuronal death in Huntington's disease. Anal. Neurol. 46, 842–849. Direct Link:
- , , , , , , , and (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.
- , , , and (2000) Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity. Nat. Genet. 25, 385–389.
- , , et al. (2002) Disruption of CREB function in brain leads to neurodegeneration. Nat. Genet. 31, 47–54.
- , and (2002) Nuclear localization of N-terminal mutant huntingtin is cell cycle dependent. Eur. J. Neurosci. 16, 355–359.
- , , , , and (2001) Histone deacetylase inhibitors reduce polyglutamine toxicity. Proc. Natl Acad. Sci. USA 98, 15179–15184.
- , , et al. (2000) CREB-binding protein sequestration by expanded polyglutamine. Hum. Mol. Genet. 9, 2197–2202.
- , , et al. (2001) Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity. Science 291, 2423–2428.
- , , et al. (2002) Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death. Neuron 34, 701–713.
- , , , , , , , , and (1997) Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333–344.
- , , , , and (1998) Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. J. Cell Biol. 143, 1457–1470.
- and (2001) Cause of neural death in neurodegenerative diseases attributable to expansion of glutamine repeats. Nature 412, 143–144.
- , , , , , and (1999) Disassembly of nuclear inclusions in the dividing cell – a novel insight into neurodegeneration. Hum. Mol. Genet. 8, 2451–2459.
- (2002) Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders. Neuron 35, 819–822.
- , and (2003) Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 421, 373–379.
- , , and (1998) Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95, 55–66.
- , , , , , , , , and (1997) Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549–558.
- , , et al. (2000) Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat. Genet. 26, 29–36.
- , , , , , , and (2000) Motoneuronal cell death is not correlated with aggregate formation of androgen receptors containing an elongated polyglutamine tract. Hum. Mol. Genet. 9, 133–144.
- , , , , , , and (2001) Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease. Hum. Mol. Genet. 10, 1307–1315.
- , , , , , , , and (1998) SH3GL3 associates with the Huntingtin exon 1 protein and promotes the formation of polygln-containing protein aggregates. Mol. Cell 2, 427–436.
- , , et al. (2001) Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 413, 739–743.
- , , , , , , , , and (2000) The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc. Natl Acad. Sci. USA 97, 6763–6768.
- , , , , , , , and (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, 731–741.
- , and (2002) Intranuclear ataxin1 inclusions contain both fast- and slow-exchanging components. Nat. Cell Biol. 4, 806–810.
- , , , , and (2001) Identities of sequestered proteins in aggregates from cells with induced polyglutamine expression. J. Cell Biol. 153, 283–294.
- , , , , and (2000) Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease. Proc. Natl Acad. Sci. USA 97, 8093–8097.
- , , , , , and (2001) Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Mol. Biol. Cell 12, 1393–1407.
- , , , , , , , and (1999) PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival. Hum. Mol. Genet. 8, 977–987.
- , , , , and (1999) Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70. Nat. Genet. 23, 425–428.
- , , , , , , , , and (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, 939–948.
- , and (2000) Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease. Cell 101, 57–66.
- , , , , and (2001) SCA7 mouse models show selective stabilization of mutant ataxin-7 and similar cellular responses in different neuronal cell types. Hum. Mol. Genet. 10, 1679–1692.
- , , , , , , , , , , et al. (2003) Huntingtin interacts with. REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes. Nat. Genet. 35, 76–83.

1471-4159/asset/olbannerleft.gif?v=1&s=4636ca86ad7e40d133e71d09a5d759010472e0c2)
1471-4159/asset/olbannerright.gif?v=1&s=38094197c2d87aeb3b86aa293b5858c5c15cca29)
