TorsinA and heat shock proteins act as molecular chaperones: Suppression of α-synuclein aggregation

Journal of Neurochemistry

Volumn 83, Issue 4, 2002, Pages 846-854

  McLean, Pamela J ^a   Kawamata, Hibiki ^a   Shariff, Saadat ^a   Hewett, Jeffrey ^a   Sharma, Nutan ^a   Ueda, Kenji ^b   Breakefield, Xandra O ^a   Hyman, Bradley T ^a  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b TOKYO METROPOLITAN INSTITUTE OF MEDICAL SCIENCE   (Japan)

Author keywords

synuclein; Aggregation; Heat shock proteins; Lewy body; Parkinson's disease; TorsinA

Indexed keywords

ADENINE; ALPHA SYNUCLEIN; CHAPERONE; GUANINE; HEAT SHOCK PROTEIN; PROTEIN DERIVATIVE; PROTEIN TORSINA; UNCLASSIFIED DRUG;

ARTICLE; CONTROLLED STUDY; DELETION MUTANT; HUMAN; HUMAN TISSUE; LEWY BODY; MOLECULAR MODEL; PARKINSON DISEASE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN CONTENT; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN LOCALIZATION;

ALPHA-SYNUCLEIN; ALZHEIMER DISEASE; CARRIER PROTEINS; CELL LINE; GENE EXPRESSION; HEAT-SHOCK PROTEINS; HUMANS; INCLUSION BODIES; LEWY BODIES; LEWY BODY DISEASE; MACROMOLECULAR SUBSTANCES; MOLECULAR CHAPERONES; NERVE TISSUE PROTEINS; PROTEIN BINDING; PROTEIN FOLDING; SUBSTANTIA NIGRA; SYNUCLEINS; TRANSFECTION;

EID: 0036846119     PISSN: 00223042     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1471-4159.2002.01190.x     Document Type: Article

References (28)

1. Arima K., Ueda K., Sunohara N., Arakawa K., Hirai S., Nakamura M., Tonozuka-Uehara H. and Kawai M. (1998) NACP/alpha-synuclein immunoreactivity in fibrillary components of neuronal and oligodendroglial cytoplasmic inclusions in the pontine nuclei in multiple system atrophy. Acta Neuropathol. (Berl.) 96, 439-444.
2. Augood S. J., Martin D. M., Ozelius L. J., Breakefield X. O., Penney J. B. Jr and Standaert D. G. (1999) Distributions of the mRNAs encoding torsinA and torsinB in the normal adult human brain. Ann. Neurol. 46, 761-769.
3. Auluck P. K., Chan H. Y. E., Trojanowski J. Q., Lee V. M.-Y. and Bonini N. M. (2002) Chaperone suppression of α-synuclein toxicity in a Drosophila model for Parkinson's disease. Science 295, 865-868.
4. Breakefield X. O., Kamm C. and Hanson P. I. (2001) TorsinA: Movement at many levels. Neuron 31, 9-12.
5. Chai Y., Koppenhafer S. L., Bonini N. M. and Paulson H. L. (1999) Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. J Neurosci. 19, 10338-10347.
6. Cummings C. J., Mancini M. A., Antalffy B., Defranco D. B., Orr H. T. and Zoghbi H. Y. (1998) Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1. Nat. Genet. 19, 148-154.
7. Cyr D. M., Langer T. and Douglas M. G. (1994) Dna-J like proteins: Molecular chaperones and specific regulators of Hsp70. Trends Biochem. Sci. 19, 176-181.
8. Engelender S., Kaminsky Z., Guo X., Sharp A. H., Amaravi R. K., Kleiderlein J. J., Margolis R. L., Troncoso J. C., Lanahan A. A., Worley P. F., Dawson V. L., Dawson T. M. and Ross C. A. (1999) Synphilin-1 associates with α-synuclein and promotes the formation of cytosolic inclusions. Nat. Genet. 22, 110-114.
9. Fink A. L. (1999) Chaperone-mediated protein folding. Physiol. Rev. 79, 425-449.
10. George J. M., Jin H., Woods W. S. and Clayton D. F. (1995) Characterization of a novel protein regulated during the critical period for song learning in the zebra finch. Neuron 15, 361-372.
11. Gomez-Tortosa E., Newell K., Irizarry M. C., Sanders J. L. and Hyman B. T. (2000) Alpha-synuclein immunoreactivity in dementia with Lewy bodies: Morphological staging and comparison with ubiquitin immunostaining. Acta Neuropathol. (Berl.) 99, 352-357.
12. Hartl F. U. (1996) Molecular chaperones in cellular protein folding. Nature 381, 571-580.
13. Hewett J., Gonzalez-Agosti C., Slater D., Ziefer P., Li S., Bergeron D., Jacoby D. J., Ozelius L. J., Ramesh V. and Breakefield X. O. (2000) Mutant torsinA, responsible for early-onset torsion dystonia, forms membrane inclusions in cultured neural cells. Hum. Mol. Genet. 9, 1403-1413.
14. Irizarry M. C., Growdon W., Gomez-Isla T., Newell K., George J. M., Clayton D. F. and Hyman B. T. (1998) Nigral and cortical lewy bodies and dystrophic nigral neurites in Parkinson's disease and cortical lewy body disease contain α-synuclein immunoreactivity. J. Neuropathol. Exp. Neurol. 57, 334-337.
15. Kawamata H., McLean P. J., Sharma N. and Hyman B. T. (2001) Interaction of α-synuclein and synphilin-1: Effect of Parkinson's disease associated muations. J. Neurochem. 77, 929-934.
16. Klein C., Friedman J., Bressman S., Vieregge P., Brin M. F., Pramstaller P. P., Leon D. D., Hagenah J., Sieberer M., Fleet C., Kiely R., Xin W., Breakefield X. O., Ozelius L. J. and Sims K. B. (1999) Genetic testing for early-onset torsion dystonia (DYT1): Introduction of a simple screening method, experiences from testing of a large cohort, and ethical aspects. Genet. Test. 3, 323-328.
17. Kobayashi Y., Kume A., Li M., Doyu M., Hata M., Ohtsuka K. and Sobue G. (2000) Chaperones Hsp70 and Hsp40 suppress aggregate formation and apoptosis in cultured neuronal cells expressing truncated androgen receptor with expanded polyglutamine tract. J. Biol. Chem. 275, 8772-8778.
18. Krüger R., Kuhn W., Müller T., Woitalla D., Graeber M., Kosel S., Przuntek H., Epplen J. T., Schols L. and Riess O. (1998) Ala30Pro mutation in the gene encoding α-synuclein in Parkinson's disease. Nat. Genet. 18, 106-108.
19. Kushnirov V. V., Kryndushkin D. S., Boguta M., Smimov V. N. and Ter-Avanesyan M. D. (2000) Chaperones that cure yeast artificial [PSI+] and their prion-specific effects. Curr. Biol. 10, 1443-1446.
20. McLean P. J., Kawamata H. and Hyman B. T. (2001) α-synuclein-enhanced green fluorescent protein fusion proteins form proteasomal sensitive inclusions in primary neurons. Neuroscience 104, 901-912.
21. Ozelius L. J., Hewett J. W., Page C. E., Bressman S. B., Kramer P. L., Shalish C., Leon D. D., Brin M. F., Raymond D., Corey D. P., Fahn S., Risch N. J., Buckler A. J., Gusella J. F. and Breakefield X. O. (1997) The early-onset torsion dystonia gene (DYT 1) encodes an ATP-binding protein. Nat. Genet. 17, 40-48.
22. Perier F., Radeke C. M., Raab-Graham K. F. and Vandenberg C. A. (1995) Expression of a putative ATPase suppresses the growth defect of a yeast potassium transport mutant: Identification of a mammalian member of the Clp/Hsp104 family. Gene 152, 157-163.
23. Polymeropoulos M. H., Lavedan C., Leroy E., Idle S. E., Dehejia A., Dutra A., Pike B., Root H., Rubenstein J., Boyer R., Stenroos E. S., Chandrasekharappa S., Athanassiadou A., Papaetropoulos T., Johnson W. G., Lazzarini A. M., Duvoisin R. C., Iorio G. D., Golbe L. I. and Nussbaum R. L. (1997) Mutation in the α-synuclein gene identified in families with Parkinson's disease. Science 276, 2045-2047.
24. Sharma N., Hewett J., Ozelius L. J., Ramesh V., McLean P. J., Breakefield X. O. and Hyman B. T. (2001) A close association of torsinA and α-synuclein in Lewy bodies: A fluorescence resonance energy transfer study. Am. J. Pathol. 159, 339-344.
25. Shashidharan P., Good P. F., Hsu A., Perl D. P., Brin M. F. and Olanow C. W. (2000) TorsinA accumulation in Lewy bodies in sporadic Parkinson's disease. Brain Res. 877, 379-381.
26. Sherman M. Y. and Goldberg A. L. (2001) Cellular defenses against unfolded proteins: A cell biologist thinks about neurodegenerative diseases. Neuron 29, 15-32.
27. Spillantini M. G., Crowther R. A., Jakes R., Hasegawa M. and Goedert M. (1998) α-synuclein in filamentous inclusions of Lewy bodies from Parkinson's disease and dementia with Lewy bodies. Proc. Natl Acad. Sci. USA 95, 6469-6473.
28. Stenoien D. L., Cummings C. J., Adams H. P., Mancini M. G., Patel K., Martino G. N., Marcelli M., Weigel N. L. and Mancini M. A. (1999) Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum. Mol. Genet. 8, 731-741.