Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch

1. La Spada, A.R., Wilson, E.M., Lubahn, D.B., Harding, A.E. & Fischbeck, K.H. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 352, 77−79 (1991). | Article | PubMed  | ChemPort |
2. The Hunington's Disese Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell72, 971−983 (1993). | PubMed  | ISI |
3. Orr, H.T. et al. Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type 1. Nature Genet. 4, 221−226 (1993). | PubMed  | ISI | ChemPort |
4. Koide, R. et al. Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nature Genet. 6, 9−13 (1994). | PubMed  | ISI | ChemPort |
5. Nagafuchi, S. et al. Expansion of an unstable CAG trinucleotide on chromosome 12p in dentatorubral and pallidoluysian atrophy. Nature Genet.6, 14−18 (1994). | PubMed  | ISI | ChemPort |
6. Kawaguchi, Y. et al. CAG repeat expansion in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nature Genet. 8, 221−227 (1994). | PubMed  | ISI | ChemPort |
7. Sanpei, K. et al. Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT. Nature Genet. 14, 277−284 (1996). | PubMed  | ISI | ChemPort |
8. Pulst, S.M. et al. Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nature Genet. 14, 269−276 (1996). | PubMed  | ISI | ChemPort |
9. Imbert, G. et al. Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats. Nature Genet. 14, 285−291 (1996). | PubMed  | ISI | ChemPort |
10. Zhuchenko, O. et al. Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha la-voltage-dependent calcium channel. Nature Genet. 15, 62−69 (1997). | PubMed  | ISI | ChemPort |
11. David, G. et al. Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion. Nature Genet. 17, 65−70 (1997). | PubMed  | ISI | ChemPort |
12. Lubahn, D.B. et al. Cloning of human androgen receptor complementary DMA and localization to the X chromosome. Science 240, 327−330 (1988). | PubMed  | ISI | ChemPort |
13. Nagafuchi, S. et al. Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA). Nature Genet. 8, 177−182 (1994). | PubMed  | ISI | ChemPort |
14. Mori, Y. et al. Primary structure and functional expression from complementary DNA of a brain calcium channel. Nature 350, 398−402 (1991). | Article | PubMed  | ISI | ChemPort |
15. Yazawa, I. et al. Abnormal gene product identified in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) brain. Nature Genet.10, 99−103 (1995). | PubMed  | ISI | ChemPort |
16. Trottier, Y. et al. Cellular localization of the huntington's disease protein and discrimination of the normal and mutated form. Nature Genet.10, 104−110 (1995). | PubMed  | ISI | ChemPort |
17. Servadio, A.. et al. Expression analysis of the ataxin-1 protein in tissues from normal and spinocerebellar ataxia type 1 individuals. Nature Genet. 10, 94−98 (1995). | PubMed  | ISI | ChemPort |
18. Onodera, O.. et al. Molecular cloning of a full-length cDNA for dentatorubral-pallidoluysian atrophy and regional expressions of the expanded alleles in the CNS. Am. J. Hum. Genet. 57, 1050−1060 (1995). | PubMed  | ISI | ChemPort |
19. Burright, E.N. et al. SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell 82, 937−948 (1995). | PubMed  | ISI | ChemPort |
20. Ikeda, H. et al. Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nature Genet. 13, 196−202 (1996). | PubMed  | ISI | ChemPort |
21. Mangiarini, L. et al Exon 1 of the HD gene with an expanded cagr repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87, 493−506 (1996). | Article | PubMed  | ISI | ChemPort |
22. Davies, S.W. et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537−548 (1997). | Article | PubMed  | ISI | ChemPort |
23. Perutz, M.F., Johnson, T., Suzuki, M. & Finch, J.T. Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proc. Natl. Acad. Sci. USA 91, 5355−5358 (1994). | PubMed  | ChemPort |
24. Stott, K., Blackburn, J.M., Butler, P.J. & Perutz, M.F. Incorporation of glutamine repeats makes protein oligomerize: implications for neurodegenerative diseases. Proc. Natal. Acad. Sci. USA 92, 6509−6513 (1995). | ChemPort |
25. Kahlem, P., Terre, C., Green, H. & Djian, P. Peptides containing glutamine repeats as substrates for transglutaminase-catalyzed cross-linking: relevance to diseases of the nervous system. Proc. Natl. Acad. Sci. USA 93, 14580−14585 (1996). | Article | PubMed  | ChemPort |
26. Naito, H. & Oyanagi, S. Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy. Neurol. 32, 798−807 (1982). | ISI | ChemPort |
27. Ikeuchi, T. et al. Dentatorubral-pallidoluysian atrophy (DRPLA): Clinical features are closely related to unstable expansions of trinucleotide (CAG) repeat. Ann. Neurol. 37, 769−775 (1995).
28. Lorand, L. et al. Specificity of guinea pig liver transglutaminase for amine substrates. Biochemistry 18, 1756−1765 (1979). | PubMed  | ISI | ChemPort |
29. Dickson, R.B., Willingham, M.C. & Pastan, I. Binding and internalization of 125l-cc2-macroglobulin by cultured fibroblast. J. Biol. Chem. 256, 3454−3459 (1981). | PubMed  | ISI | ChemPort |
30. Kleman, J.-P., Aeschlimann, D., Paulsson, M. & van der Rest, M. Transglutaminase-catalyzed cross linking of fibrils of collagen V/XI in A 204 rhabdomyosarcoma cell. Biochemistry 34, 13768−13775 (1995). | PubMed  | ISI | ChemPort |
31. Paulson, H.L. et al. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333−334 (1997). | PubMed  | ISI | ChemPort |
32. Scherzinger, E. et al. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549−558 (1997). | Article | PubMed  | ISI | ChemPort |
33. Onodera, O. et al. Oligomerization of expanded-polyglutamine domain fluorescent fusion proteins in cultured mammalian cells. Biochem.Biophy. Res. Commun. 238, 599−605 (1997). | Article | ChemPort |
34. Jackson, M., et al. The cortical neuritic pathology of Huntington's disease. Neuropatho. Appl. Neurobiol. 21, 18−26 (1995). | ISI | ChemPort |
35. DiFiglia, M. et al. Aggregation of Huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990−1993 (1997). | Article | PubMed  | ISI | ChemPort |
36. Paulson, H.L. et al. Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain. Ann. Neurol. 41, 453−462 (1997). | PubMed  | ISI | ChemPort |
37. Goldberg, Y.P. et al. Cleavage of huntingtin by apopain, aproapoptotic cysteine protease, is modulated by the polyglutamine tract. Nature Genet. 13, 442−449 (1996). | PubMed  | ISI | ChemPort |
38. Mizushima, S. & Nagata, S.. pEF-BOS, a powerful mammalian expression vector. Nucleic Acids Res.18, 5322 (1990). | PubMed  | ISI | ChemPort |

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