Protein degradation, aggregation, and misfolding

Movement Disorders

Volumn 25, Issue SUPPL. 1, 2010, Pages

  Cuervo, Ana Maria ^a   Wong, Esther S P ^a   Martinez Vicente, Marta ^a  

^a ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

Author keywords

Autophagy; Lysosomes; Neurodegeneration; Proteases; Protein aggregates

Indexed keywords

PROTEASOME; UBIQUITIN; ALPHA SYNUCLEIN; PROTEIN;

ARTICLE; AUTOPHAGY; NERVE DEGENERATION; PARKINSON DISEASE; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN DEGRADATION; QUALITY CONTROL; ANIMAL; CHEMICAL STRUCTURE; HUMAN; METABOLISM; NERVE CELL; PATHOLOGY; PHYSIOLOGY; PROTEIN CONFORMATION; PROTEIN FOLDING; REVIEW;

ALPHA-SYNUCLEIN; ANIMALS; AUTOPHAGY; HUMANS; MODELS, MOLECULAR; NEURONS; PARKINSON DISEASE; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEIN CONFORMATION; PROTEIN FOLDING; PROTEINS; UBIQUITIN;

EID: 77953463398     PISSN: 08853185     EISSN: 15318257     Source Type: Journal    
DOI: 10.1002/mds.22718     Document Type: Article

References (31)

1. Finkbeiner S, Cuervo A, Morimoto R, Muchowski P. Disease-modifying pathways in neurodegeneration. J Neurosci 2006; 26: 10349-10357.
2. Kopito R. Aggresomes, inclusion bodies and protein aggregation. Trends Cell Biol 2000; 10: 523-530.
3. Webb JL, Ravikumar B, Rubinsztein DC. Microtubule disruption inhibits autophagosome-lysosome fusion: implications for studying the roles of aggresomes in polyglutamine diseases. Int J Biochem Cell Biol 2004; 36: 2541-2550.
4. Hara T, Nakamura K, Matsui M, et al. Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice. Nature 2006; 441: 885-889.
5. Komatsu M, Waguri S, Chiba T, et al. Loss of autophagy in the central nervous system causes neurodegeneration in mice. Nature 2006; 441: 880-884.
6. Gidalevitz T, Ben-Zvi A, Ho K, Brignull H, Morimoto R. Progressive disruption of cellular protein folding in models of polyglutamine diseases. Science 2006; 311: 1471-1474.
7. Mizushima N, Klionsky D. Protein turnover via autophagy: implications for metabolism. Annu Rev Nutr 2007; 27: 19-40.
8. Cuervo A. Autophagy: in sickness and in health. Trends Cell Biol 2004; 14: 70-77.
9. Martinez-Vicente M, Cuervo AM. Autophagy and neurodegeneration: when the cleaning crew goes on strike. Lancet Neurol 2007; 6: 352-361.
10. Bennett EJ, Bence NF, Jayakumar R, Kopito RR. Global impairment of the ubiquitin-proteasome system by nuclear or cytoplasmic protein aggregates precedes inclusion body formation. Mol Cell 2005; 4: 351-365.
11. Webb JL, Ravikumar B, Atkins J, Skepper JN, Rubinsztein DC. Alpha-Synuclein is degraded by both autophagy and the proteasome. J Biol Chem 2003; 278: 25009-25013.
12. Cuervo AM, Stefanis L, Fredenburg R, Lansbury PT, Sulzer D. Impaired degradation of mutant alpha-synuclein by chaperone-mediated autophagy. Science 2004; 305: 1292-1295.
13. Martinez-Vicente M, Talloczy Z, Kaushik S, et al. Dopamine modified α-synuclein blocks chaperone-mediated autophagy. JClin Invest 2008; 118: 777-788.
14. McNaught KS, Perl DP, Brownell AL, Olanow CW. Systemic exposure to proteasome inhibitors causes a progressive model of Parkinson's disease. Ann Neurol 2004; 56: 149-162.
15. Marx FP, Soehn AS, Berg D, et al. The proteasomal subunit S6 ATPase is a novel synphilin-1 interacting protein-implications for Parkinson's disease. FASEB J 2007; 21: 1759-1767.
16. Leroy E, Boyer R, Auburger G, et al. The ubiquitin pathway in Parkinson's disease. Nature 1998: 395: 451-452.
17. Tan JM, Wong ES, Kirkpatrick DS, et al. Lysine 63-linked ubiquitination promotes the formation and autophagic clearance of protein inclusions associated with neurodegenerative diseases. Hum Mol Genet 2008; 17: 431-439.
18. Lim K, Chew K, Tan J, et al. Parkin mediates nonclassical, proteasomal-dependent ubiquitination of synphilin-1: implications for Lewy body formation. J Neurosci 2005; 25: 2002-2009.
19. Liu Y, Fallon L, Lashuel HA, Liu Z, Lansbury JPT. The UCH-L1 gene encodes two opposing enzymatic activities that affect α-synuclein degradation and Parkinson's disease susceptibility. Cell 2002; 111: 209-218.
20. Klionsky DJ. The molecular machinery of autophagy: unanswered questions. J Cell Sci 2005; 118(Pt 1): 7-18.
21. Massey A, Kiffin R, Cuervo AM. Pathophysiology of chaperone-mediated autophagy. Int J Biochem Cell Biol 2004; 36: 2420-2434.
22. Shintani T, Klionsky DJ. Autophagy in health and disease: a double-edged sword. Science 2004; 306: 990-995.
23. Dice J. Chaperone-mediated autophagy. Autophagy 2007; 3: 295-299.
24. Massey AC, Kaushik S, Sovak G, Kiffin R, Cuervo AM. Consequences of the selective blockage of chaperone-mediated autophagy. Proc Nat Acad Sci U S A 2006; 103: 5905-5910.
25. Iwata A, Riley BE, Johnston JA, Kopito RR. HDAC6 and microtubules are required for autophagic degradation of aggregated huntingtin. J Biol Chem 2005; 280: 40282-40292.
26. Ravikumar B, Vacher C, Berger Z, et al. Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease. Nat Genet 2004; 36: 585-595.
27. Wong ES, Tan JM, Suong WE, et al. Autophagy-mediated clearance of aggresomes is not a universal phenomenon. Hum Mol Genet 2008; 17: 2570-2582.
28. Cuervo AM, Bergamini E, Brunk UT, Droge W, Ffrench M, Terman A. Autophagy and aging: the importance of maintaining "clean" cells. Autophagy 2005; 1: 131-140.
29. Kiffin R, Bandyopadhyay U, Cuervo AM. Oxidative stress and autophagy. Antioxid Redox Signal 2006; 8: 152-162.
30. Terman A, Brunk UT. Lipofuscin. Int J Biochem Cell Biol 2004; 36: 1400-1404.
31. Rubinsztein DC, DiFiglia M, Heintz N, et al. Autophagy and its possible roles in nervous system diseases, damage and repair. Autophagy 2005; 1: 11-22.