Transgenic models of α-synuclein pathology: Past, present, and future

Annals of the New York Academy of Sciences

Volumn 991, Issue , 2003, Pages 171-188

  Hashimoto, Makoto ^a   Rockenstein, Edward ^a   Masliah, Eliezer ^a,b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Transgenic models; synclein

Indexed keywords

ALPHA SYNUCLEIN; AMYLOID BETA PROTEIN; CHAPERONE; HEAT SHOCK PROTEIN 70; VIRUS VECTOR;

ANIMAL CELL; CONFERENCE PAPER; DIFFUSE LEWY BODY DISEASE; DRUG ACCUMULATION; GENE MUTATION; GENE OVEREXPRESSION; HISTOPATHOLOGY; NERVE DEGENERATION; NONHUMAN; OXIDATIVE STRESS; PARKINSON DISEASE; PROTEIN FOLDING; PROTEIN METABOLISM; PROTEIN PROCESSING; SYNAPTIC TRANSMISSION; TRANSGENIC ANIMAL;

ANIMALIA; MUS MUSCULUS;

EID: 0038389601     PISSN: 00778923     EISSN: None     Source Type: Book Series    
DOI: 10.1111/j.1749-6632.2003.tb07475.x     Document Type: Conference Paper

References (83)

1. KOO, E., P.J. LANSBURY & J. KELLY. 1999. Amyloid diseases: abnormal protein aggregation in neurodegeneration. Proc. Natl. Acad. Sci. USA 96: 9989-9990.
2. FERRIGNO, P. & P. SILVER. 2000. Polyglutamine expansions: proteolysis, chaperones, and the dangers of promiscuity. Neuron 26: 9-12.
3. RAMASSAMY, C., et al. 1999. Oxidative damage and protection by antioxidants in the frontal cortex of Alzheimer's disease is related to the apolipoprotein E genotype. Free Rad. Biol. Med. 27: 544-553.
4. SPILLANTINI, M, et al. 1997. α-Synuclein in Lewy bodies. Nature 388: 839-840.
5. TAKEDA, A., et al. 1998. Abnormal accumulation of NACP/α-synuclein in neurodegenerative disorders. Am. J. Pathol. 152: 367-372.
6. IRIZARRY, M., et al. 1998. Nigral and cortical Lewy bodies and dystrophic nigral neurites in Parkinson's disease and cortical Lewy body disease contain alpha-synuclein immunoreactivity. J. Neuropathol. Exp. Neurol. 57: 334-337.
7. WALSH, D., et al. 2000. The oligomerization of amyloid beta-protein begins intracellularly in cells derived from human brain. Biochemistry 39: 10831-10839.
8. SELKOE, D.J., et al. 1996. The role of APP processing and trafficking pathways in the formation of amyloid beta-protein. Ann. N.Y. Acad. Sci. 777: 57-64.
9. TROJANOWSKI, J.Q. & V.M. LEE. 2000. "Fatal attractions" of proteins: a comprehensive hypothetical mechanism underlying Alzheimer's disease and other neurodegenerative disorders. Ann. N.Y. Acad. Sci. 924: 62-67.
10. MUCHOWSKI, P.J. 2002. Protein misfolding, amyloid formation, and neurodegeneration: a critical role for molecular chaperones? Neuron 35: 9-12.
11. CUMMINGS, C.J. & H.Y. ZOGHBI. 2000. Trinucleotide repeats: mechanisms and pathophysiology. Annu. Rev. Genomics Hum. Genet. 1: 281-328.
12. MASLIAH, E. 2000. The role of synaptic proteins in Alzheimer's disease. Ann. N.Y. Acad. Sci. 924: 68-75.
13. MASLIAH, E. 2001. Recent advances in the understanding of the role of synaptic proteins in Alzheimer's disease and other neurodegenerative disorders. J. Alz. Dis. 3: 1-9.
14. VOLLES, M.J. & P.T. LANSBURY, JR. 2002. Vesicle permeabilization by protofibrillar alpha-synuclein is sensitive to Parkinson's disease-linked mutations and occurs by a pore-like mechanism. Biochemistry 41: 4595-4602.
15. VOLLES, M.J., et al. 2001. Vesicle permeabilization by protofibrillar alpha-synuclein: implications for the pathogenesis and treatment of Parkinson's disease. Biochemistry 40: 7812-7819.
16. LANSBURY, P.T.J. 1999. Evolution of amyloid: what normal protein folding may tell us about fibrillogenesis and disease. Proc. Natl. Acad. Sci. USA 96: 3342-3344.
17. IWAI, A. 2000. Properties of NACP/alpha-synuclein and its role in Alzheimer's disease. Biochim. Biophys. Acta 1502: 95-109.
18. MASLIAH, E., et al. 1996. Altered presynaptic protein NACP is associated with plaque formation and neurodegeneration in Alzheimer's disease. Am. J. Pathol. 148: 201-210.
19. UEDA, K., et al. 1993. Novel amyloid component (NAC) differentiates Alzheimer's disease from normal aging plaques. Soc. Neurosci. Abstr. 19: 1254.
20. JAKES, R., M. SPILLANTINI & M. GOEDERT. 1994. Identification of two distinct synucleins from human brain. FEBS Lett. 345: 27-32.
21. NAKAJO, S., et al. 1993. A new brain-specific 14-kDa protein is a phosphoprotein: its complete amino acid sequence and evidence for phosphorylation. Eur. J. Biochem. 217: 1057-1063.
22. JIA, T., et al. 1999. Stimulation of breast cancer invasion and metastasis by synuclein gamma. Cancer Res. 59: 742-747.
23. SURGUCHOV, A., et al. 1999. Synoretin - a new protein belonging to the synuclein family. Mol. Cell. Neurosci. 13: 95-103.
24. HASHIMOTO, M. & E. MASLIAH. 1999. Alpha-synuclein in Lewy body disease and Alzheimer's disease. Brain Pathol. 9: 707-720.
25. TROJANOWSKI, J. & V. LEE. 1998. Aggregation of neurofilament and alpha-synuclein proteins in Lewy bodies: implications for pathogenesis of Parkinson disease and Lewy body dementia. Arch. Neurol. 55: 151-152.
26. TAKEDA, A., et al. 1998. Abnormal distribution of the non-Aβ component of Alzheimer's disease amyloid precursor/α-synuclein in Lewy body disease as revealed by proteinase K and formic acid pretreatment. Lab. Invest. 78: 1169-1177.
27. WAKABAYASHI, K., et al. 1997. Neurofibrillary tangles in the dentate granule cells in Alzheimer's disease, Lewy body disease and progressive supranuclear palsy. Acta Neuropathol. 93: 7-12.
28. KRUGER, R., et al. 1998. Ala30Pro mutation in the gene encoding α-synuclein in Parkinsons's disease. Nat. Genet. 18: 106-108.
29. POLYMEROPOULOS, M., et al. 1997. Mutation in the α-synuclein gene identified in families with Parkinson's disease. Science 276: 2045-2047.
30. MASLIAH, E., et al. 2000. Dopaminergic loss and inclusion body formation in alpha-synuclein mice: implications for neurodegenerative disorders. Science 287: 1265-1269.
31. FEANY, M. & W. BENDER. 2000. A Drosophila model of Parkinson's disease. Nature 404: 394-398.
32. HASHIMOTO, M., et al. 1998. Human recombinant NACP/α-synuclein is aggregated and fibrillated in vitro: relevance for Lewy body disease. Brain Res. 799: 301-306.
33. CONWAY, K.A., et al. 2000. Acceleration of oligomerization, not fibrillization, is a shared property of both alpha-synuclein mutations linked to early-onset Parkinson's disease: implications for pathogenesis and therapy. Proc. Natl. Acad. Sci. USA 97: 571-576.
34. ROCHET, J., K. CONWAY & P.J. LANSBURY. 2000. Inhibition of fibrillization and accumulation of prefibrillar oligomers in mixtures of human and mouse alpha-synuclein. Biochemistry 39: 10619-10626.
35. PERRIN, R., et al. 2000. Interaction of human alpha-synuclein and Parkinson's disease variants with phospholipids: structural analysis using site-directed mutagenesis. J. Biol. Chem. 275: 34393-34398.
36. NARAYANAN, V. & S. SCARLATA. 2001. Membrane binding and self-association of alpha-synucleins. Biochemistry 40: 9927-9934.
37. WOOD, S.J., et al. 1999. alpha-Synuclein fibrillogenesis is nucleation dependent: implications for the pathogenesis of Parkinson's disease. J. Biol. Chem. 274: 19509-19512.
38. SERPELL, L., et al. 2000. Fiber diffraction of synthetic α-synuclein filaments shows amyloid-like cross-β conformation. Proc. Natl. Acad. Sci. USA 97: 4897-4902.
39. CONWAY, K., J. HARPER & P. LANSBURY. 1998. Accelerated in vitro fibril formation by a mutant alpha-synuclein linked to early-onset Parkinson disease. Nat. Med. 4: 1318-1320.
40. NARHI, L., et al. 1999. Both familial Parkinson's disease mutations accelerate alpha-synuclein aggregation. J. Biol. Chem. 274: 9843-9846.
41. HASHIMOTO, M., et al. 1999. Oxidative stress induces amyloid-like aggregate formation of NACP/α-synuclein in vitro. Neuroreport 10: 717-721.
42. HASHIMOTO, M., et al. 1999. Role of cytochrome c as a stimulator of α-synuclein aggregation in Lewy body disease. J. Biol. Chem. 274: 28849-28852.
43. HSU, L.J., et al. 2000. α-Synuclein promotes mitochondrial deficiencies and oxidative stress. Am. J. Pathol. 157: 401-410.
44. PAIK, S.R., et al. 1999. Copper(II)-induced self-oligomerization of alpha-synuclein. Biochem. J. 340: 821-828.
45. SOUZA, J., et al. 2000. Dityrosine cross-linking promotes formation of stable alpha-synuclein polymers. J. Biol. Chem. 275: 18344-18349.
46. OKOCHI, M., et al. 2000. Constitutive phosphorylation of the Parkinson's disease associated α-synuclein. J. Biol. Chem. 275: 390-397.
47. FUJIWARA, H., et al. 2002. alpha-Synuclein is phosphorylated in synucleinopathy lesions. Nat. Cell Biol. 4: 160-164.
48. JO, E., et al. 2000. Alpha-synuclein membrane iteractions and lipid specificity. J. Biol. Chem. 275: 34328-34334.
49. DAVIDSON, W., et al. 1998. Stabilization of alpha-synuclein secondary structure upon binding to synthetic membranes. J. Biol. Chem. 273: 9443-9449.
50. JENSEN, P., et al. 1997. Binding of Aβ to α- and β-synucleins: identification of segments in α-synuclein/NAC precursor that bind Aβ and NAC. Biochem. J. 323: 539-546.
51. PARK, S., et al. 1998. Self-oligomerization of NACP, the precursor protien of the non-amyloid beta/A4 protein (A beta) component of Alzheimer's disease amyloid, observed in the presence of a C-terminal A beta fragment (residues 25-35). FEBS Lett. 421: 73-76.
52. YOSHIMOTO, M., et al. 1995. NACP, the precursor protein of non-amyloid β/A4 protein (Aβ) component of Alzheimer disease amyloid, binds Aβ and stimulates Aβ aggregation. Proc. Natl. Acad. Sci. USA 92: 9141-9145.
53. HASHIMOTO, M., et al. 2001. beta-Synuclein inhibits alpha-synuclein aggregation: a possible role as an anti-parkinsonian factor. Neuron 32: 213-223.
54. BETARBET, R., T.B. SHERER & J.T. GREENAMYRE. 2002. Animal models of Parkinson's disease. Bioessays 24: 308-318.
55. BEAL, M.F. 2001. Experimental models of Parkinson's disease. Nat. Rev. Neurosci. 2: 325-334.
56. DAWSON, T., A. MANDIR & M. LEE. 2002. Animal models of PD: pieces of the same puzzle? Neuron 35: 219-222.
57. ROCKENSTEIN, E., et al. 2002. Differential neuropathological alterations in transgenic mice expressing alpha-synuclein from the platelet-derived growth factor and Thy-1 promoters. J. Neurosci. Res. 68: 568-578.
58. GIASSON, B.I., et al. 2002. Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein. Neuron 34: 521-533.
59. LEE, M.K., et al. 2002. Human alpha-synuclein-harboring familial Parkinson's disease-linked Ala-53→Thr mutation causes neurodegenerative disease with alpha-synuclein aggregation in transgenic mice. Proc. Natl. Acad. Sci. USA 99: 8968-8973.
60. VAN DER PUTTEN, H., et al. 2000. Neuropathology in mice expressing human alpha-synuclein. J. Neurosci. 20: 6021-6029.
61. RICHFIELD, E.K., et al. 2002. Behavioral and neurochemical effects of wild-type and mutated human alpha-synuclein in transgenic mice. Exp. Neurol. 175: 35-48.
62. LO BIANCO, C., et al. 2002. alpha-Synucleinopathy and selective dopaminergic neuron loss in a rat lentiviral-based model of Parkinson's disease. Proc. Natl. Acad. Sci. USA 99: 10813-10818.
63. KIRIK, D., et al. 2002. Parkinson-like neurodegeneration induced by targeted overexpression of alpha-synuclein in the nigrostriatal system. J. Neurosci. 22: 2780-2791.
64. KLEIN, R.L., et al. 2002. Dopaminergic cell loss induced by human A30P alpha-synuclein gene transfer to the rat substantia nigra. Hum. Gene Ther. 13: 605-612.
65. KAHLE, P.J., et al. 2001. Selective insolubility of alpha-synuclein in human Lewy body diseases is recapitulated in a transgenic mouse model. Am. J. Pathol. 159: 2215-2225.
66. GOEDERT, M. 2001. Alpha-synuclein and neurodegenerative diseases. Nat. Rev. Neurosci. 2: 492-501.
67. CONWAY, K.A., et al. 2001. Kinetic stabilization of the alpha-synuclein protofibril by a dopamine-alpha-synuclein adduct. Science 294: 1346-1349.
68. GIASSON, B.I., et al. 2000. Oxidative damage linked to neurodegeneration by selective alpha-synuclein nitration in synucleinopathy lesions. Science 290: 985-989.
69. LANGSTON, J.W., E.B. LANGSTON & I. IRWIN. 1984. MPTP-induced parkinsonism in human and non-human primates - clinical and experimental aspects. Acta Neurol. Scand. Suppl. 100: 49-54.
70. GRUNBLATT, E., S. MANDEL & M.B. YOUDIM. 2000. MPTP and 6-hydroxydopamine-induced neurodegeneration as models for Parkinson's disease: neuroprotective strategies. J. Neurol. 247(Suppl. 2): 95-102.
71. RATHKE-HARTLIEB, S., et al. 2001. Sensitivity to MPTP is not increased in Parkinson's disease-associated mutant alpha-synuclein transgenic mice. J. Neurochem. 77: 1181-1184.
72. SONG et al. 2002. MPTP promotes mitochondrial pathology and aggregation in human α-synuclein transgenic mice. Soc. Neurosci. Abstr. 226.1.
73. MASLIAH, E., et al. 2001. beta-Amyloid peptides enhance alpha-synuclein accumulation and neuronal deficits in a transgenic mouse model linking Alzheimer's disease and Parkinson's disease. Proc. Natl. Acad. Sci. USA 98: 12245-12250.
74. HANSEN, L., et al. 1990. The Lewy body variant of Alzheimer's disease: A clinical and pathologic entity. Neurology 40: 1-7.
75. MCKEITU, I., et al. 1996. Clinical and pathological diagnosis of dementia with Lewy bodies (DLB): Report of the CDLB International Workshop. Neurology 47: 1113-1124.
76. AULUCK, P.K., et al. 2002. Chaperone suppression of alpha-synuclein toxicity in a Drosophila model for Parkinson's disease. Science 295: 865-868.
77. HATTORI, N., et al. 1998. Point mutations (Thr240Arg and Ala311Stop) in the Parkin gene. Biochem. Biophys. Res. Commun. 249: 754-758.
78. KITADA, T., et al. 1998. Mutations in the Parkin gene cause autosomal recessive juvenile parkinsonism. Nature 392: 605-608.
79. SHIMURA, H., et al. 2000. Familial Parkinson disease gene product, Parkin, is a ubiquitin-protein ligase. Nat. Gen. 25: 302-305.
80. WEINREB, P., et al. 1996. NACP, a protein implicated in Alzheimer's disease and learning, is natively unfolded. Biochemistry 35: 13709-13715.
81. UVERSKY, V.N., et al. 2002. Biophysical properties of the synucleins and their propensities to fibrillate: inhibition of alpha-synuclein assembly by beta- and gamma-synucleins. J. Biol. Chem. 277: 11970-11978.
82. LEE, H.J., et al. 2001. Formation and removal of alpha-synuclein aggregates in cells exposed to mitochondrial inhibitors. J. Biol. Chem. 277: 5411-5417.
83. LENG, Y., T.N. CHASE & M.C. BENNETT. 2001. Muscarinic receptor stimulation induces translocation of an alpha-synuclein oligomer from plasma membrane to a light vesicle fraction in cytoplasm. J. Biol. Chem. 276: 28212-28218.