Evidence for a recruitment and sequestration mechanism in Huntington's disease

Philosophical Transactions of the Royal Society B: Biological Sciences

Volumn 354, Issue 1386, 1999, Pages 1029-1034

  Preisinger, Elizabeth ^a   Jordan, Barbara M ^a   Kazantsev, Aleksey ^a   Housman, David ^a  

^a MASSACHUSETTS INSTITUTE OF TECHNOLOGY   (United States)

Author keywords

Aggregation; Huntington's disease; Neurodegeneration; Polyglutamine; Trinucleotide; Triplet repeats

Indexed keywords

EID: 0033614768     PISSN: 09628436     EISSN: None     Source Type: Journal    
DOI: 10.1098/rstb.1999.0455     Document Type: Article

References (13)

1. Cha, J. H., Kosinski, C. M., Kerner, J. A., Alsdorf, S. A., Mangiarini, L., Davies, S.W., Penney, J. B., Bates, G. P. & Young, A. B. 1998 Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene. Proc. Natl Acad. Sci. USA 95, 6480-6485.
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 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. Klement, I. A., Skinner, P. J., Kaytor, M. D., Yi, H., Hersch, S. M., Clark, H. B., Zoghbi, H.Y. & Orr, H.T. 1998 Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice. Cell 95, 41-53.
5. Kosinski, C. M., Cha, J. H., Young, A. B., Persichetti, F., MacDonald, M., Gusella, J. F., Penney Jr, J. B. & Standaert, D. G. 1997 Huntingtin immunoreactivity in the rat neostriatum: differential accumulation in projection and interneurons. Exp. Neurol. 144, 239-247.
6. Li, S. H. & Li, X. J. 1998 Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats. Hum. Mol. Genet. 7, 777-782.
7. Ordway, J. M. (and 11 others) 1997 Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse. Cell 91, 753-763.
8. 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. & Pittman, R. N. 1997 Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron 19, 333-344.
9. Perutz, M. F., Johnson, T., Suzuki, M. & Finch, J. T. 1994 Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proc. Natl Acad. Sci. USA 91, 5355-5358.
10. Reddy, P. S. & Housman, D. E. 1997 The complex pathology of trinucleotide repeats. Curr. Opin. Cell Biol. 9, 364-372.
11. Scherzinger, E., Lurz., R., Turmaine, M., Mangiarini, L., Hollenbach, R., 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.
12. Vonsattel, J. P., Myers, R. H., Stevens, T. J., Ferrante, R. J., Bird, E. D. & Richardson Jr, E. P. 1985 Neuropathological classification of Huntington's disease. J. Neuropathol. Exp. Neurol. 44, 559-577.
13. Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N. & Bonini, N. M. 1998 Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell 93, 939-949.