Ubiquilin overexpression reduces GFP-polyalanine-induced protein aggregates and toxicity

Experimental Cell Research

Volumn 313, Issue 13, 2007, Pages 2810-2820

  Wang, Hongmin ^a   Monteiro, Mervyn J ^a  

^a UNIVERSITY OF MARYLAND BIOTECHNOLOGY INSTITUTE   (United States)

Author keywords

Polyalanine; Polyglutamine; Ubiquilin; Ubiquitin

Indexed keywords

ALANINE; GREEN FLUORESCENT PROTEIN; POLYALANINE; POLYGLUTAMINE; PROTEIN DERIVATIVE; SMALL INTERFERING RNA; UBIQUILIN; UBIQUITIN; UNCLASSIFIED DRUG;

ARTICLE; CELL DEATH; CELL PROLIFERATION; CONTROLLED STUDY; CYTOTOXICITY; HELA CELL; HUMAN; HUMAN CELL; IMMUNOPRECIPITATION; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN INDUCTION;

EID: 34447096237     PISSN: 00144827     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.yexcr.2007.04.006     Document Type: Article

References (36)

1. Pearson C.E., Nichol Edamura K., and Cleary J.D. Repeat instability: mechanisms of dynamic mutations. Nat. Rev., Genet. 6 (2005) 729-742
2. Gatchel J.R., and Zoghbi H.Y. Diseases of unstable repeat expansion: mechanisms and common principles. Nat. Rev., Genet. 6 (2005) 743-755
3. Bates G. Huntingtin aggregation and toxicity in Huntington's disease. Lancet 361 (2003) 1642-1644
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. Brown L.Y., and Brown S.A. Alanine tracts: the expanding story of human illness and trinucleotide repeats. Trends Genet. 20 (2004) 51-58
6. Amiel J., Trochet D., Clement-Ziza M., Munnich A., and Lyonnet S. Polyalanine expansions in human. Hum. Mol. Genet. 13 Spec No 2 (2004) R235-R243
7. Albrecht A., and Mundlos S. The other trinucleotide repeat: polyalanine expansion disorders. Curr. Opin. Genet. Dev. 15 (2005) 285-293
8. Rankin J., Wyttenbach A., and Rubinsztein D.C. Intracellular green fluorescent protein-polyalanine aggregates are associated with cell death. Biochem. J. 348 Pt. 1 (2000) 15-19
9. Nasrallah I.M., Minarcik J.C., and Golden J.A. A polyalanine tract expansion in Arx forms intranuclear inclusions and results in increased cell death. J. Cell Biol. 167 (2004) 411-416
10. Berger Z., Davies J.E., Luo S., Pasco M.Y., Majoul I., O'Kane C.J., and Rubinsztein D.C. Deleterious and protective properties of an aggregate-prone protein with a polyalanine expansion. Hum. Mol. Genet. 15 (2006) 453-465
11. 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
12. 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
13. Ko H.S., Uehara T., and Nomura Y. Role of ubiquilin associated with protein-disulfide isomerase in the endoplasmic reticulum in stress-induced apoptotic cell death. J. Biol. Chem. 277 (2002) 35386-35392
14. Massey L.K., Mah A.L., and Monteiro M.J. Ubiquilin regulates presenilin endoproteolysis and modulates gamma-secretase components, Pen-2 and nicastrin. Biochem. J. 391 (2005) 513-525
15. Bao Y.P., Cook L.J., O'Donovan D., Uyama E., and Rubinsztein D.C. Mammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophy. J. Biol. Chem. 277 (2002) 12263-12269
16. Berger Z., Ravikumar B., Menzies F.M., Oroz L.G., Underwood B.R., Pangalos M.N., Schmitt I., Wullner U., Evert B.O., O'Kane C.J., and Rubinsztein D.C. Rapamycin alleviates toxicity of different aggregate-prone proteins. Hum. Mol. Genet. 15 (2006) 433-442
17. Michalik A., and Van Broeckhoven C. Pathogenesis of polyglutamine disorders: aggregation revisited. Hum. Mol. Genet. 12 Spec No 2 (2003) R173-R186
18. La Spada A.R., and Taylor J.P. Polyglutamines placed into context. Neuron 38 (2003) 681-684
19. Ross C.A., and Poirier M.A. Protein aggregation and neurodegenerative disease. Nat. Med. 10 Suppl. (2004) S10-S17
20. Arrasate M., Mitra S., Schweitzer E.S., Segal M.R., and Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 431 (2004) 805-810
21. Bowman A.B., Yoo S.Y., Dantuma N.P., and Zoghbi H.Y. Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin-proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation. Hum. Mol. Genet. 14 (2005) 679-691
22. 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
23. 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
24. 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
25. 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
26. Wu S., Mikhailov A., Kallo-Hosein H., Hara K., Yonezawa K., and Avruch J. Characterization of ubiquilin 1, an mTOR-interacting protein. Biochim. Biophys. Acta 1542 (2002) 41-56
27. Kaye F.J., Modi S., Ivanovska I., Koonin E.V., Thress K., Kubo A., Kornbluth S., and Rose M.D. A family of ubiquitin-like proteins binds the ATPase domain of Hsp70-like Stch. FEBS Lett. 467 (2000) 348-355
28. 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
29. Ford D.L., and Monteiro M.J. Dimerization of ubiquilin is dependent upon the central region of the protein: evidence that the monomer, but not the dimer, is involved in binding presenilins. Biochem. J. 399 (2006) 397-404
30. Thomas A.V., Herl L., Spoelgen R., Hiltunen M., Jones P.B., Tanzi R.E., Hyman B.T., and Berezovska O. Interaction between presenilin 1 and ubiquilin 1 as detected by fluorescence lifetime imaging microscopy and a high-throughput fluorescent plate reader. J. Biol. Chem. 281 (2006) 26400-26407
31. Bertram L., Hiltunen M., Parkinson M., Ingelsson M., Lange C., Ramasamy K., Mullin K., Menon R., Sampson A.J., Hsiao M.Y., Elliott K.J., Velicelebi G., Moscarillo T., Hyman B.T., Wagner S.L., Becker K.D., Blacker D., and Tanzi R.E. Family-based association between Alzheimer's disease and variants in UBQLN1. N. Engl. J. Med. 352 (2005) 884-894
32. Brouwers N., Sleegers K., Engelborghs S., Bogaerts V., van Duijn C.M., De Deyn P.P., Van Broeckhoven C., and Dermaut B. The UBQLN1 polymorphism, UBQ-8i, at 9q22 is not associated with Alzheimer's disease with onset before 70 years. Neurosci. Lett. 392 (2006) 72-74
33. Kamboh M.I., Minster R.L., Feingold E., and DeKosky S.T. Genetic association of ubiquilin with Alzheimer's disease and related quantitative measures. Mol. Psychiatry 11 (2006) 273-279
34. Slifer M.A., Martin E.R., Bronson P.G., Browning-Large C., Doraiswamy P.M., Welsh-Bohmer K.A., Gilbert J.R., Haines J.L., and Pericak-Vance M.A. Lack of association between UBQLN1 and Alzheimer disease. Am. J. Med. Genet., B Neuropsychiatr. Genet. 141 (2006) 208-213
35. Smemo S., Nowotny P., Hinrichs A.L., Kauwe J.S., Cherny S., Erickson K., Myers A.J., Kaleem M., Marlowe L., Gibson A.M., Hollingworth P., O'Donovan M.C., Morris C.M., Holmans P., Lovestone S., Morris J.C., Thal L., Li Y., Grupe A., Hardy J., Owen M.J., Williams J., and Goate A. Ubiquilin 1 polymorphisms are not associated with late-onset Alzheimer's disease. Ann. Neurol. 59 (2006) 21-26
36. Perry R.T., Wiener H., Harrell L.E., Blacker D., Tanzi R.E., Bertram L., Bassett S.S., and Go R.C. Follow-up mapping supports the evidence for linkage in the candidate region at 9q22 in the NIMH Alzheimer's disease Genetics Initiative cohort. Am. J. Med. Genet. B Neuropsychiatr. Genet. 144 (2007) 220-227