Excess α-synuclein worsens disease in mice lacking ubiquitin carboxy-terminal hydrolase L1

Scientific Reports

Volumn 2, Issue , 2012, Pages

  Shimshek, Derya R ^a   Schweizer, Tatjana ^a   Schmid, Peter ^a   Van Der Putten, P Herman ^a  

^a NOVARTIS PHARMA AG   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA SYNUCLEIN; PRIMER DNA; UBIQUITIN CARBOXYL TERMINAL HYDROLASE L 1, MOUSE; UBIQUITIN CARBOXYL-TERMINAL HYDROLASE L-1, MOUSE; UBIQUITIN THIOLESTERASE;

ANIMAL; ARTICLE; ENZYMOLOGY; GENETICS; IMMUNOHISTOCHEMISTRY; METABOLISM; MOUSE; NUCLEOTIDE SEQUENCE; PARKINSON DISEASE; PATHOPHYSIOLOGY; TRANSGENIC MOUSE;

ALPHA-SYNUCLEIN; ANIMALS; BASE SEQUENCE; DNA PRIMERS; IMMUNOHISTOCHEMISTRY; MICE; MICE, TRANSGENIC; PARKINSON DISEASE; UBIQUITIN THIOLESTERASE;

EID: 84859776138     PISSN: None     EISSN: 20452322     Source Type: Journal    
DOI: 10.1038/srep00262     Document Type: Article

References (60)

1. Esposito, E., Di Matteo, V. & Di Giovanni, G. Death in the substantia nigra: A motor tragedy. Expert Rev Neurother 7, 677-697 (2007). (Pubitemid 46911617)
2. Dev, K. K., Hofele, K., Barbieri, S., Buchman, V. L. & van der Putten, H. Part II: Alpha-synuclein and its molecular pathophysiological role in neurodegenerative disease. Neuropharmacology 45, 14-44 (2003). (Pubitemid 36695009)
3. Vanderhaeghen, J. J., Perier, O. & Sternon, J. E. Pathological findings in idiopathic orthostatic hypotension. Its relationship with Parkinson's disease. Arch Neurol 22, 207-214 (1970).
4. Bennett, M. C. The role of alpha-synuclein in neurodegenerative diseases. Pharmacol Ther 105, 311-331 (2005).
5. Spillantini, M. G. et al. Alpha-synuclein in Lewy bodies. Nature 388, 839-840 (1997).
6. Takeda, A. et al. Abnormal accumulation of NACP/alpha-synuclein in neurodegenerative disorders. Am J Pathol 152, 367-372 (1998). (Pubitemid 28106382)
7. Wakabayashi, K.,Matsumoto, K., Takayama, K., Yoshimoto, M. & Takahashi, H. NACP, a presynaptic protein, immunoreactivity in Lewy bodies in Parkinson's disease. Neurosci Lett 239, 45-48 (1997). (Pubitemid 28055846)
8. Kruger, R. et al. Ala30Pro mutation in the gene encoding alpha-synuclein in Parkinson's disease. Nat Genet 18, 106-108 (1998).
9. Polymeropoulos, M. H. et al. Mutation in the alpha-synuclein gene identified in families with Parkinson's disease. Science 276, 2045-2047 (1997). (Pubitemid 27443610)
10. Singleton,A. B. et al. alpha-Synuclein locus triplication causes Parkinson's disease. Science 302, 841 (2003). (Pubitemid 37339619)
11. Zarranz, J. J. et al. The new mutation, E46K, of alpha-synuclein causes Parkinson and Lewy body dementia. Ann Neurol 55, 164-173 (2004). (Pubitemid 38166992)
12. Feany, M. B. & Bender, W. W. A Drosophila model of Parkinson's disease. Nature 404, 394-398 (2000). (Pubitemid 30164341)
13. Kirik, D. et al. Nigrostriatal alpha-synucleinopathy induced by viral vectormediated overexpression of human alpha-synuclein: A new primate model of Parkinson's disease. Proc Natl Acad Sci U S A 100, 2884-2889 (2003). (Pubitemid 36297593)
14. Lee, M. K. et al. Human alpha-synuclein-harboring familial Parkinson's diseaselinked Ala-53 -> Thr mutation causes neurodegenerative disease with alphasynuclein aggregation in transgenic mice. Proc Natl Acad Sci U S A 99, 8968-8973 (2002). (Pubitemid 34693667)
15. Masliah, E. et al. Dopaminergic loss and inclusion body formation in alphasynuclein mice: Implications for neurodegenerative disorders. Science 287, 1265-1269 (2000). (Pubitemid 30112148)
16. van der Putten, H. et al. Neuropathology in mice expressing human alphasynuclein. J Neurosci 20, 6021-6029 (2000). (Pubitemid 30658395)
17. Ardley, H. C. & Robinson, P. A. The role of ubiquitin-protein ligases in neurodegenerative disease. Neurodegener Dis 1, 71-87 (2004). (Pubitemid 39369421)
18. Yamazaki, T. et al. PGP9.5 as a marker for invasive colorectal cancer. Clin Cancer Res 8, 192-195 (2002). (Pubitemid 34101477)
19. Larsen, C. N., Price, J. S. & Wilkinson, K. D. Substrate binding and catalysis by ubiquitin C-terminal hydrolases: Identification of two active site residues. Biochemistry 35, 6735-6744 (1996). (Pubitemid 26172432)
20. Wilkinson, K. D. et al. The neuron-specific protein PGP 9.5 is a ubiquitin carboxyl-terminal hydrolase. Science 246, 670-673 (1989). (Pubitemid 19283361)
21. Liu, Y., Fallon, L., Lashuel, H. A., Liu, Z. & Lansbury, P. T., Jr. The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility. Cell 111, 209-218 (2002). (Pubitemid 35292441)
22. Osaka, H. et al. Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron. Hum Mol Genet 12, 1945-1958 (2003). (Pubitemid 37038804)
23. Lowe, J., McDermott, H., Landon, M., Mayer, R. J. &Wilkinson, K. D. Ubiquitin carboxyl-terminal hydrolase (PGP 9.5) is selectively present in ubiquitinated inclusion bodies characteristic of human neurodegenerative diseases. J Pathol 161, 153-160 (1990). (Pubitemid 20195792)
24. Leroy, E. et al. The ubiquitin pathway in Parkinson's disease. Nature 395, 451-452 (1998).
25. Healy, D. G. et al. UCHL-1 is not a Parkinson's disease susceptibility gene. Ann Neurol 59, 627-633 (2006).
26. Maraganore, D. M. et al. UCHL1 is a Parkinson's disease susceptibility gene. Ann Neurol 55, 512-521 (2004). (Pubitemid 38391968)
27. Satoh, J.& Kuroda, Y. Apolymorphic variation of serine to tyrosine at codon 18 in the ubiquitin C-terminal hydrolase-L1 gene is associated with a reduced risk of sporadic Parkinson's disease in a Japanese population. J Neurol Sci 189, 113-117 (2001). (Pubitemid 32776657)
28. Ichihara, N. et al. Axonal degeneration promotes abnormal accumulation of amyloid beta-protein in ascending gracile tract of gracile axonal dystrophy (GAD) mouse. Brain Res 695, 173-178 (1995).
29. Saigoh, K. et al. Intragenic deletion in the gene encoding ubiquitin carboxyterminal hydrolase in gad mice. Nat Genet 23, 47-51 (1999). (Pubitemid 29418786)
30. Wang, Y. L. et al. Accumulation of beta- and gamma-synucleins in the ubiquitin carboxyl-terminal hydrolase L1-deficient gad mouse. Brain Res 1019, 1-9 (2004).
31. Walters, B. J. et al. Differential effects of Usp14 and Uch-L1 on the ubiquitin proteasome system and synaptic activity. Mol Cell Neurosci 39, 539-548 (2008).
32. Chen, F., Sugiura, Y.,Myers, K. G., Liu, Y. & Lin, W. Ubiquitin carboxyl-terminal hydrolase L1 is required for maintaining the structure and function of the neuromuscular junction. Proc Natl Acad Sci U S A 107, 1636-1641.
33. Yasuda, T. et al. Effects of UCH-L1 on alpha-synuclein over-expression mouse model of Parkinson's disease. J Neurochem 108, 932-944 (2009).
34. Kabuta, T., Furuta, A., Aoki, S., Furuta, K. & Wada, K. Aberrant interaction between Parkinson disease-associated mutant UCH-L1 and the lysosomal receptor for chaperone-mediated autophagy. J Biol Chem 283, 23731-23738 (2008).
35. Setsuie, R. et al. Dopaminergic neuronal loss in transgenic mice expressing the Parkinson's disease-associated UCH-L1 I93M mutant. Neurochem Int 50, 119-129 (2007). (Pubitemid 44879668)
36. Imai, Y., Soda, M. & Takahashi, R. Parkin suppresses unfolded protein stressinduced cell death through its E3 ubiquitin-protein ligase activity. J Biol Chem 275, 35661-35664 (2000).
37. Sampathu, D. M., Giasson, B. I., Pawlyk, A. C., Trojanowski, J. Q. & Lee, V. M. Ubiquitination of alpha-synuclein is not required for formation of pathological inclusions in alpha-synucleinopathies. Am J Pathol 163, 91-100 (2003). (Pubitemid 36759594)
38. Barrachina, M. et al. Reduced ubiquitin C-terminal hydrolase-1 expression levels in dementia with Lewy bodies. Neurobiol Dis 22, 265-273 (2006).
39. Choi, J. et al. Oxidative modifications and down-regulation of ubiquitin carboxylterminal hydrolase L1 associated with idiopathic Parkinson's and Alzheimer's diseases. J Biol Chem 279, 13256-13264 (2004). (Pubitemid 38445905)
40. Liu, Z. et al. Membrane-associated farnesylated UCH-L1 promotes alphasynuclein neurotoxicity and is a therapeutic target for Parkinson's disease. Proc Natl Acad Sci U S A 106, 4635-4640 (2009).
41. Rieker, C. et al. Neuropathology in mice expressing mouse alpha-synuclein. PLoS One 6, e24834 (2011).
42. Yamazaki, K. et al.Gracile axonal dystrophy (GAD), a new neurological mutant in the mouse. Proc Soc Exp Biol Med 187, 209-215 (1988). (Pubitemid 18077798)
43. Shimshek, D. R.,Mueller, M.,Wiessner, C., Schweizer, T. & van der Putten, P. H. The HSP70 molecular chaperone is not beneficial in a mouse model of alphasynucleinopathy. PLoS One 5, e10014.
44. Larsen, C. N., Krantz, B. A. & Wilkinson, K. D. Substrate specificity of deubiquitinating enzymes: Ubiquitin C-terminal hydrolases. Biochemistry 37, 3358-3368 (1998). (Pubitemid 28125563)
45. McNaught, K. S., Belizaire, R., Isacson, O., Jenner, P. & Olanow, C. W. Altered proteasomal function in sporadic Parkinson's disease. Exp Neurol 179, 38-46 (2003). (Pubitemid 36062617)
46. McNaught, K. S. & Olanow, C. W. Proteolytic stress: A unifying concept for the etiopathogenesis of Parkinson's disease. Ann Neurol 53 Suppl 3, S73-84; discussion S84-76 (2003).
47. Olanow, C.W. & McNaught, K. S. Ubiquitin-proteasome system and Parkinson's disease. Mov Disord 21, 1806-1823 (2006). (Pubitemid 44856478)
48. Kurihara, L. J., Kikuchi, T.,Wada, K. & Tilghman, S. M. Loss of Uch-L1 and Uch- L3 leads to neurodegeneration, posterior paralysis and dysphagia. Hum Mol Genet 10, 1963-1970 (2001). (Pubitemid 32910541)
49. Hashimoto, M., Rockenstein, E., Mante, M., Mallory, M. & Masliah, E. beta- Synuclein inhibits alpha-synuclein aggregation: A possible role as an antiparkinsonian factor. Neuron 32, 213-223 (2001). (Pubitemid 33030190)
50. Cartier, A. E. et al. Regulation of synaptic structure by ubiquitin C-terminal hydrolase L1. J Neurosci 29, 7857-7868 (2009).
51. Cullen, V. et al. Cathepsin D expression level affects alpha-synuclein processing, aggregation, and toxicity in vivo. Mol Brain 2, 5 (2009).
52. Adamczyk, A. & Strosznajder, J. B. Alpha-synuclein potentiates Ca2+ influx through voltage-dependent Ca2+ channels. Neuroreport 17, 1883-1886 (2006). (Pubitemid 46006291)
53. Liu, S. et al. alpha-Synuclein produces a long-lasting increase in neurotransmitter release. Embo J 23, 4506-4516 (2004). (Pubitemid 39601975)
54. Chandra, S., Gallardo, G., Fernandez-Chacon, R., Schluter, O. M. & Sudhof, T. C. Alpha-synuclein cooperates with CSPalpha in preventing neurodegeneration. Cell 123, 383-396 (2005). (Pubitemid 41546670)
55. Ballanyi, K., Grafe, P. & ten Bruggencate, G. Ion activities and potassium uptake mechanisms of glial cells in guinea-pig olfactory cortex slices. J Physiol 382, 159-174 (1987). (Pubitemid 17226141)
56. Montgomery, D. L. Astrocytes: Form, functions, and roles in disease. Vet Pathol 31, 145-167 (1994).
57. Shimshek, D. R. et al. Forebrain-specific glutamate receptor B deletion impairs spatial memory but not hippocampal field long-term potentiation. J Neurosci 26, 8428-8440 (2006). (Pubitemid 44316171)
58. Galter, D., Westerlund, M., Belin, A. C. & Olson, L. DJ-1 and UCH-L1 gene activity patterns in the brains of controls, Parkinson and schizophrenia patients and in rodents. Physiol Behav (2007).
59. Chen, Y. et al. Astrocytes protect neurons from nitric oxide toxicity by a glutathione-dependent mechanism. J Neurochem 77, 1601-1610 (2001). (Pubitemid 32574495)
60. Saura, J. et al. Intranigral infusion of interleukin-1beta activates astrocytes and protects from subsequent 6-hydroxydopamine neurotoxicity. J Neurochem 85, 651-661 (2003). (Pubitemid 36531928)