Ubiquilin interacts and enhances the degradation of expanded-polyglutamine proteins

Biochemical and Biophysical Research Communications

Volumn 360, Issue 2, 2007, Pages 423-427

  Wang, Hongmin ^a   Monteiro, Mervyn J ^a  

^a UNIVERSITY OF MARYLAND BIOTECHNOLOGY INSTITUTE   (United States)

Author keywords

Aggregation; Huntington's disease; Polyglutamine; Protein turnover; Ubiquilin

Indexed keywords

GREEN FLUORESCENT PROTEIN; HUNTINGTIN; HYBRID PROTEIN; POLYGLUTAMINE; UBIQUILIN; UNCLASSIFIED DRUG;

ARTICLE; EXON; HUMAN; IMMUNOFLUORESCENCE MICROSCOPY; IMMUNOPRECIPITATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN BINDING; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN INTERACTION; PROTEIN LOCALIZATION; PROTEIN METABOLISM;

CARRIER PROTEINS; CELL CYCLE PROTEINS; HUMANS; PEPTIDES;

EID: 34447095637     PISSN: 0006291X     EISSN: 10902104     Source Type: Journal    
DOI: 10.1016/j.bbrc.2007.06.097     Document Type: Article

References (18)

1. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group, Cell 72 (1993) 971-983.
2. Bates G. Huntingtin aggregation and toxicity in Huntington's disease. Lancet 361 (2003) 1642-1644
3. Wexler N.S., Lorimer J., Porter J., Gomez F., Moskowitz C., Shackell E., Marder K., Penchaszadeh G., Roberts S.A., Gayan J., Brocklebank D., Cherny S.S., Cardon L.R., Gray J., Dlouhy S.R., Wiktorski S., Hodes M.E., Conneally P.M., Penney J.B., Gusella J., Cha J.H., Irizarry M., Rosas D., Hersch S., Hollingsworth Z., MacDonald M., Young A.B., Andresen J.M., Housman D.E., De Young M.M., Bonilla E., Stillings T., Negrette A., Snodgrass S.R., Martinez-Jaurrieta M.D., Ramos-Arroyo M.A., Bickham J., Ramos J.S., Marshall F., Shoulson I., Rey G.J., Feigin A., Arnheim N., Acevedo-Cruz A., Acosta L., Alvir J., Fischbeck K., Thompson L.M., Young A., Dure L., O'Brien C.J., Paulsen J., Brickman A., Krch D., Peery S., Hogarth P., Higgins Jr. D.S., and Landwehrmeyer B. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. Proc. Natl. Acad. Sci. USA 101 (2004) 3498-3503
4. Riley B.E., and Orr H.T. Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle. Genes Dev. 20 (2006) 2183-2192
5. In: Bates G., Harper P., and Jones L. (Eds). Huntington's Disease. third ed. (2002), Oxford University Press, Oxford
6. Michalik A., and Van Broeckhoven C. Pathogenesis of polyglutamine disorders: aggregation revisited. Hum. Mol. Genet. 12 Spec. No. 2 (2003) R173-R186
7. Doi H., Mitsui K., Kurosawa M., Machida Y., Kuroiwa Y., and Nukina N. Identification of ubiquitin-interacting proteins in purified polyglutamine aggregates. FEBS Lett. 571 (2004) 171-176
8. Mah A.L., Perry G., Smith M.A., and Monteiro M.J. Identification of ubiquilin, a novel presenilin interactor that increases presenilin protein accumulation. J. Cell. Biol. 151 (2000) 847-862
9. Elsasser S., and Finley D. Delivery of ubiquitinated substrates to protein-unfolding machines. Nat. Cell. Biol. 7 (2005) 742-749
10. Wu A.L., Wang J., Zheleznyak A., and Brown E.J. Ubiquitin-related proteins regulate interaction of vimentin intermediate filaments with the plasma membrane. Mol. Cell 4 (1999) 619-625
11. Conklin D., Holderman S., Whitmore T.E., Maurer M., and Feldhaus A.L. Molecular cloning, chromosome mapping and characterization of UBQLN3 a testis-specific gene that contains an ubiquitin-like domain. Gene 249 (2000) 91-98
12. Kleijnen M.F., Shih A.H., Zhou P., Kumar S., Soccio R.E., Kedersha N.L., Gill G., and Howley P.M. The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome. Mol. Cell. 6 (2000) 409-419
13. Kleijnen M.F., Alarcon R.M., and Howley P.M. The ubiquitin-associated domain of hPLIC-2 interacts with the proteasome. Mol. Biol. Cell. 14 (2003) 3868-3875
14. Massey L.K., Mah A.L., Ford D.L., Miller J., Liang J., Doong H., and Monteiro M.J. Overexpression of ubiquilin decreases ubiquitination and degradation of presenilin proteins. J. Alzheimers Dis. 6 (2004) 79-92
15. Wang H., Lim P.J., Yin C., Rieckher M., Vogel B.E., and Monteiro M.J. Suppression of polyglutamine-induced toxicity in cell and animal models of Huntington's disease by ubiquilin. Hum. Mol. Genet. 15 (2006) 1025-1041
16. Ko H.S., Uehara T., Tsuruma K., and Nomura Y. Ubiquilin interacts with ubiquitylated proteins and proteasome through its ubiquitin-associated and ubiquitin-like domains. FEBS Lett. 566 (2004) 110-114
17. Raasi S., Varadan R., Fushman D., and Pickart C.M. Diverse polyubiquitin interaction properties of ubiquitin-associated domains. Nat. Struct. Mol. Biol. 12 (2005) 708-714
18. Wang H., and Monteiro M.J. Ubiquilin overexpression reduces GFP-polyalanine-induced protein aggregates and toxicity. Exp. Cell. Res. (2007)