Aggregation of truncated GST-HD exon 1 fusion proteins containing normal range and expanded glutamine repeats

Philosophical Transactions of the Royal Society B: Biological Sciences

Volumn 354, Issue 1386, 1999, Pages 991-994

  Hollenbach, B ^a   Scherzinger, E ^a   Schweiger, K ^a   Lurz, R ^a   Lehrach, H ^a   Wanker, E E ^a  

^a MAX PLANCK INSTITUTE FOR MOLECULAR GENETICS   (Germany)

Author keywords

Aggregation; Amyloid fibrillogenesis; Glutamine repeat; Huntingtin; Huntington's disease

Indexed keywords

ALZHEIMER DISEASE; AMYLOID BETA PROTEIN; CARBOXY TERMINAL SEQUENCE; ELECTRON MICROSCOPY; EXON; GLUTAMINE; GLUTATHIONE TRANSFERASE; HUNTINGTON CHOREA; HYBRID PROTEIN; MOLECULAR WEIGHT; OLIGOMER; PROTEIN AGGREGATION; PROTEIN HYDROLYSIS; PROTEINASE;

EID: 0033614778     PISSN: 09628436     EISSN: None     Source Type: Journal    
DOI: 10.1098/rstb.1999.0450     Document Type: Article

References (18)

1. Becher, M. W., Kotzuk, J. A., Sharp, A. H., Davies, S. W., Bates, G. P., Price, D. L. & Ross, C. A. 1998 Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length. Neurobiol. Dis. 4, 387-395.
2. Davies, S. W., Turmaine, M., Cozens, B. A., DiFiglia, M., Sharp, A. H., Ross, C. A., Scherzinger, E., Wanker, E. E., Mangiarini, L. & Bates, G. P. 1997 Formation of neuronal intranuclear inclusions (NII) underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90, 537-548.
3. DiFiglia, M., Sapp, E.; Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P. & Aronin, N. 1997 Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277, 1990-1993.
4. Harper, P. S. 1996 Huntington's disease, 2nd edn. London: W. B. Saunders Co.
5. Holmberg, M. (and 10 others) 1998 Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions. Hum. Mol. Genet. 7, 913-918.
6. Huntington's Disease Collaborative Research Group 1993 A novel gene containing a trinucleotide repeat that is unstable on Huntington's disease chromosomes. Cell 72, 971-983.
7. Igarashi, S. (and 18 others) 1998 Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nature Genet. 18, 111-117.
8. Mangiarini, L. (and 10 others) 1996 Exon 1 of the Huntington's disease gene containing a highly expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87, 493-506.
9. Matilla, A., Koshy, B. T., Cummings, C. J., Isobe, T., Orr, H. T. & Zoghbi, H. Y. 1997 The cerebellar leucine-rich acidic nuclear protein interacts with ataxin-1. Nature 389, 974-978.
10. Paulson, H. L., Perez, M. K., Tottier, Y., Trojanowski, J. Q., Subramony, S. H., Das, S. S., Vig, P., Mandel, J.-L., Fischbeck, K. H. & Pittman, R. N. 1997 Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333-344.
11. Perutz, M. 1996 Glutamine repeats and inherited neurodegenerative diseases: molecular aspects. Curr. Opin. Struct. Biol. 6, 848-858.
12. Rubinsztein, D. C. (and 36 others) 1996 Phenotypic characterisation of individuals with 30-40 CAG repeats in the Huntington's disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats. Am. J. Hum. Genet. 59, 16-22.
13. 180 repeat expansion and the propagation of highly expanded CAG repeats in lambda phage. Hum. Genet. 99, 692-695.
14. Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H. & Wanker, E. E. 1997 Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90, 549-558.
15. Skinner, P. J., Koshy, B. T., Cummings, C. L., Klement, I. A., Helin, K., Servadio, A., Zoghbi, H. Y. & Orr, H. T. 1997 Ataxin-1 with an expanded glutamine tract alters nuclear matrix-associated structures. Nature 389, 971-974.
16. Smith, D. B. & Johnson, K. S. 1988 Single-step purification of peptides expressed in Escherichia coli as fusions with glutathione S-transferase. Gene 67, 31-40.
17. Vonsattel, J.-P., Myers, R. H., Stevens, T. J., Ferrante, R. J., Bird, E. D. & Richardson, E. P. 1985 Neuropathological classification of Huntington's disease. J. Neuropath. Exp. Neurol. 44, 559-577.
18. Wanker, E. E., Scherzinger, E., Heiser, V., Sittler, A., Eickhoff, H. & Lehrach, H. 1999 Membrane filter assay for the detection of amyloid-like protein aggregates. Methods Enzymol. (In the press.)