Modest proteasomal inhibition by aberrant ubiquitin exacerbates aggregate formation in a Huntington disease mouse model

Molecular and Cellular Neuroscience

Volumn 43, Issue 3, 2010, Pages 281-286

  de Pril, Remko ^a   Hobo, Barbara ^a   van Tijn, Paula ^a   Roos, Raymund A C ^b   van Leeuwen, Fred W ^a   Fischer, David F ^a  

^a NETHERLANDS INSTITUTE FOR NEUROSCIENCE   (Netherlands)

^b LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

Huntingtin; Inclusions; Neurodegeneration; Polyglutamine disease; Transgenic mice; Ubiquitin proteasome system

Indexed keywords

HUNTINGTIN; LENTIVIRUS VECTOR; NEURON SPECIFIC NUCLEAR PROTEIN; POLYGLUTAMINE; PROTEASOME; UBIQUITIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; CORPUS STRIATUM; HUNTINGTON CHOREA; MALE; MOUSE; NEUROPATHOLOGY; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; TRANSGENIC MOUSE;

ANIMALS; CELL DEATH; DISEASE MODELS, ANIMAL; HUNTINGTON DISEASE; MALE; MICE; MICE, TRANSGENIC; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; PEPTIDES; PROTEASOME ENDOPEPTIDASE COMPLEX; UBIQUITIN;

MUS; MUS MUSCULUS;

EID: 75849128321     PISSN: 10447431     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.mcn.2009.12.001     Document Type: Article

References (23)

1. 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
2. Bence N.F., Sampat R.M., and Kopito R.R. Impairment of the ubiquitin-proteasome system by protein aggregation. Science 292 (2001) 1552-1555
3. Bennett E.J., Shaler T.A., Woodman B., et al. Global changes to the ubiquitin system in Huntington's disease. Nature 448 (2007) 704-708
4. Bett J.S., Cook C., Petrucelli L., and Bates G.P. The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease. PLoS ONE e5128 (2009) 4
5. de Pril R., Fischer D.F., Maat-Schieman M.L.C., et al. Accumulation of aberrant ubiquitin induces aggregate formation and cell death in polyglutamine diseases. Hum. Mol. Genet. 13 (2004) 1803-1813
6. De Vrij F.M., Sluijs J.A., Gregori L., et al. Mutant ubiquitin expressed in Alzheimer's disease causes neuronal death. FASEB J. 15 (2001) 2680-2688
7. DiFiglia M., Sapp E., Chase K.O., et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277 (1997) 1990-1993
8. Fischer D.F., de Vos R.A.I., van Dijk R., et al. Disease-specific accumulation of mutant ubiquitin as a marker for proteasomal dysfunction in the brain. FASEB J. 17 (2003) 2014-2024
9. Fischer D.F., van Dijk R., van Tijn P., et al. Long-term proteasome dysfunction in the mouse brain by expression of aberrant ubiquitin. Neurobiol. Aging 30 (2009) 847-863
10. Halliday G.M., McRitchie D.A., Macdonald V., Double K.L., Trent R.J., and McCusker E. Regional specificity of brain atrophy in Huntington's disease. Exp. Neurol. 154 (1998) 663-672
11. Maat-Schieman M.L., Dorsman J.C., Smoor M.A., et al. Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain. J. Neuropathol. Exp. Neurol. 58 (1999) 129-137
12. McNeil S., Novelletto A., Srinidhi J., et al. Reduced penetrance of the Huntington's disease mutation. Hum. Mol. Genet. 6 (1997) 775-779
13. Pandey U.B., Nie Z., Batlevi Y., et al. HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature 447 (2007) 859-863
14. Paxinos G., and Franklin K.B.J. The mouse brain in stereotaxic coordinates. Academic, San Diego, Calif. London xxv (2001) 264
15. Ross C.A., and Poirier M.A. Protein aggregation and neurodegenerative disease. Nat. Med. 10 Suppl (2004) S10-S17
16. Schmidt T., Lindenberg K.S., Krebs A., et al. Protein surveillance machinery in brains with spinocerebellar ataxia type 3: redistribution and differential recruitment of 26S proteasome subunits and chaperones to neuronal intranuclear inclusions. Ann. Neurol. 51 (2002) 302-310
17. Schwartz A.L., and Ciechanover A. Targeting proteins for destruction by the ubiquitin system: implications for human pathobiology. Annu. Rev. Pharmacol. Toxicol. 49 (2009) 73-96
18. Seo H., Sonntag K.C., and Isacson O. Generalized brain and skin proteasome inhibition in Huntington's disease. Ann. Neurol. 56 (2004) 319-328
19. Trottier Y., Lutz Y., Stevanin G., et al. Polyglutamine expansion as a pathological epitope in Huntington's disease and four dominant cerebellar ataxias. Nature 378 (1995) 403-406
20. van Tijn P., de Vrij F.M.S., Schuurman K.G., et al. Dose-dependent inhibition of proteasome activity by a mutant ubiquitin associated with neurodegenerative disease. J. Cell. Sci. 120 (2007) 1615-1623
21. Wexler N.S., Lorimer J., Porter J., et al. Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset. PNAS 101 (2004) 3498-3503
22. Yoshizawa T., Yoshida H., and Shoji S. Differential susceptibility of cultured cell lines to aggregate formation and cell death produced by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch. Brain Res. Bull. 56 (2001) 349-352
23. Zoghbi H.Y., and Orr H.T. Glutamine repeats and neurodegeneration. Annu. Rev. Neurosci. 23 (2000) 217-247