Recruitment and the role of nuclear localization in polyglutamine- mediated aggregation

Journal of Cell Biology

Volumn 143, Issue 6, 1998, Pages 1457-1470

  Perez, Matthew K ^a   Paulson, Henry L ^b   Pendse, Sagun J ^a   Saionz, Sarah J ^a   Bonini, Nancy M ^a   Pittman, Randall N ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b UNIVERSITY OF IOWA   (United States)

Author keywords

Hereditary ataxia; Machado Joseph disease; Neurodegeneration; Trinucleotide; Triplet repeat

Indexed keywords

GREEN FLUORESCENT PROTEIN; HYBRID PROTEIN;

ARTICLE; CELL NUCLEUS; CONTROLLED STUDY; DROSOPHILA; HUMAN; HUMAN TISSUE; IMMUNOBLOTTING; IMMUNOFLUORESCENCE MICROSCOPY; IMMUNOHISTOCHEMISTRY; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN ANALYSIS; SPINOCEREBELLAR DEGENERATION; TRANSGENE;

ANIMALS; ANIMALS, GENETICALLY MODIFIED; CELL NUCLEUS; DNA-BINDING PROTEINS; DROSOPHILA; DROSOPHILA PROTEINS; EYE PROTEINS; HUMANS; INCLUSION BODIES; MACHADO-JOSEPH DISEASE; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; PEPTIDE FRAGMENTS; PEPTIDES; RECOMBINANT FUSION PROTEINS; REPRESSOR PROTEINS; TATA BOX; TATA-BOX BINDING PROTEIN; TRANSCRIPTION FACTORS; TRANSFECTION;

ATAXIA;

EID: 0032517816     PISSN: 00219525     EISSN: None     Source Type: Journal    
DOI: 10.1083/jcb.143.6.1457     Document Type: Article

References (19)

1. Banif, S., A. Servadio, M. Chung, T.J. Kwiatkowski, A.E. McCall, L.A. Duvick, Y. Shen, E.J. Roth, H.T. Orr, and H.Y. Zoghbi. 1994. Identification and characterization of the gene causing type 1 spinocerebellar ataxia. Nat. Genet. 7:513-520.
2. Bonini, N.M., W.M. Leiserson, and S. Benzer. 1993. The eyes absent gene: genetic control of cell survival and differentiation in the developing Drosophila eye. Cell. 72:379-395.
3. Bonini, N.M., W.M. Leiserson, and S. Benzer. 1998. Expression and multiple roles of the eyes absent gene in Drosophila. Dev. Biol. 129:42-57.
4. Burright, E.N., H.B. Clark, A. Servadio, T. Matilla, R.M. Feddersen, W.S. Yunis, L.A. Duvick, H.Y. Zoghbi, and H.T. Orr. 1995. SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat. Cell. 82:937-948.
5. Cooper, J.K., G. Schilling, M.F. Peters, W.J. Herring, A.H. Sharp, Z. Kaminsky, J. Masone, F.A. Khan, M. Delanoy, D.R. Borchelt, et al. 1998. Truncated N-terminal fragments of huntingtin with expanded glutamine repeats form nuclear and cytoplasmic aggregates in cell culture. Hum. Mol. Genet. 7:783-790.
6. Cummings, C.J., M.A. Mancini, B. Antalffy, D.B. DeFranco, H.T. Orr, and H.Y. Zoghbi. 1998. Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet. 19:148-154.
7. Davies, S.W., K. Beardsall, M. Turmaine, M. DiFiglia, N. Aronin, and G.P. Bates. 1998. Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansion. Lancet. 351:131-133.
8. DiFiglia, M., E. Sapp, K.O. Chase, S.W. Davies, J.P. Vonsattel, and N. Aronin. 1997. Aggregation of Huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science. 277:1990-1993.
9. Durr, A., G. Stevanin, G. Cancel, C. Duyckaerts, N. Abbas, O. Didierjcan, H. Chneiweiss, A. Benomar, O. Lyon-Caen, J. Julien, et al. 1996. Spinocerebellar ataxia type 3 and Machado-Joseph disease: clinical, molecular and neuropathologic features. Ann. Neural. 39:490-499.
10. Gerber, H.-P., K. Seipel, O. Georgiev, M. Hofferer, M. Hug, S. Rusconi, and W. Schaffner. 1994. Transcriptional activation modulated by homopolymeric glutamine and proline stretches. Science. 263:808-811.
11. Glover, J.R., and S. Lindquist. 1998. Hsp104, hsp70, and hsp40: a novel chaperone system that rescues previously aggregated proteins. Cell. 94:73-82.
12. Goldberg, Y.P., D.W. Nicholson, D.M. Rasper, M.A. Kalchman, H.B. Koide, R.K. Graham, M. Bromm, P. Kazemi-Esfarjani, N.A. Thornberry, J.P. Vaillancourt, and M.R. Hayden. 1996. Cleavage of huntingtin by apopain, a proapoptotic cystine protease, is modulated by the polyglutamine tract. Nat. Genet. 13:442-449.
13. Haekam, A.S., C.L. Wellington, and M.R. Hayden. 1998. The fatal attraction of polyglutamine-containing proteins. Clin. Genet. 53:233-242.
14. Holmberg, M., C. Duyckaerts, A. Durr, G. Cancel, I. Gourfinkel-An, P. Damier, B. Faucheux, Y. Trottier, E.C. Hirsch, Y. Agid, and A. Brice. 1998. Spinocerebellar ataxia type 7 (SCA7): a neurodegenerative disorder with neuronal intranuclear inclusions. Hum. Mol. Genet. 7:913-918.
15. Igarashi, S., R. Koide, T. Shimohata, M. Yamada, Y. Hayashi, H. Takano, H. Date, M. Oyake, T. Sato, A. Sato, et al. 1998. Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. Nat. Genet. 18:111-117.
16. Ikeda, H., M. Yamaguchi, S. Sugai, Y. Aze, S. Narumiya, and A. Kakizuka. 1996. Expanded polyglutamine in the Machado-Joseph disease protein induces cell death in vitro and in vivo. Nat. Genet. 13:196-202.
17. Kahlem, P., C. Terre, H. Green, and P. Djian. 1996. 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.
18. Kawaguchi, Y., T. Okamoto, M. Taniwaki, M. Aizawa, M. Inoue, S. Katayama, H. Kawakami, S. Nakamura, M. Nishimura, I. Akiguchi, et al. 1994. CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat. Genet. 8:221-228.
19. Klement, I.A., P.J. Skinner, M.D. Kaytor, H. Yi, S.M. Hersch, H.B. Clark, H.Y. Zoghbi, and H.T. Orr. 1998. Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in transgenic mice. Cell. 95:41-53.