Cross-functional E3 ligases Parkin and C-terminus Hsp70-interacting protein in neurodegenerative disorders

Journal of Neurochemistry

Volumn 120, Issue 3, 2012, Pages 350-370

  Kumar, Pravir ^a,b   Pradhan, Kaveri ^a   Karunya, R ^a   Ambasta, Rashmi K ^a   Querfurth, Henry W ^c  

^a VIT UNIVERSITY   (India)

^b TUFTS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c BROWN UNIVERSITY   (United States)

Author keywords

CHIP; Neurodegeneration; Parkin; Proteinopathy; Ubiquitin E3 Ligase

Indexed keywords

ALPHA SYNUCLEIN; AMYLOID BETA PROTEIN; AMYLOID BETA PROTEIN[1-42]; CARBOXY TERMINUS HEAT SHOCK PROTEIN 70 INTERACTING PROTEIN; CHAPERONE; INSULINASE; MEMBRANE METALLOENDOPEPTIDASE; PARKIN; POLYUBIQUITIN; PROTEASOME; UBIQUITIN PROTEIN LIGASE E3; UNCLASSIFIED DRUG;

ALZHEIMER DISEASE; AMYGDALOID NUCLEUS; AMYOTROPHIC LATERAL SCLEROSIS; AUTOPHAGY; AUTOSOMAL RECESSIVE INHERITANCE; BLOOD BRAIN BARRIER; CATALYSIS; CELL DEATH; CELL PROTECTION; COGNITIVE DEFECT; DISEASE MODEL; ENERGY METABOLISM; GENE; GENE MUTATION; HUMAN; HUNTINGTON CHOREA; LYSOSOME; MOTOR DYSFUNCTION; NERVE FIBER TRANSPORT; NONHUMAN; OXIDATIVE STRESS; PARKIN GENE; PARKINSON DISEASE; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN FUNCTION; PROTEIN PROCESSING; PROTEIN TRANSPORT; REVIEW; SYNAPSE; TAUOPATHY; TRANSCYTOSIS;

AMYLOID BETA-PEPTIDES; ANIMALS; HUMANS; MODELS, BIOLOGICAL; NEURODEGENERATIVE DISEASES; UBIQUITIN-PROTEIN LIGASE COMPLEXES; UBIQUITIN-PROTEIN LIGASES;

ANIMALIA;

EID: 84855695865     PISSN: 00223042     EISSN: 14714159     Source Type: Journal    
DOI: 10.1111/j.1471-4159.2011.07588.x     Document Type: Review

References (279)

1. Almeida A., Bolaños J. P., and, Moreno S., (2005) Cdh1/Hct1-APC is essential for the survival of postmitotic neurons. J. Neurosci. 25, 8115-8121. (Pubitemid 41292106)
2. Al-Ramahi I., Lam Y. C., Chen H. K., et al. (2006) CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J. Biol. Chem. 281, 26714-26724. (Pubitemid 44401880)
3. Aravind L., and, Koonin E. V., (2000) The U box is a modified RING finger-a common domain in ubiquitination. Curr. Biol. 10, R132-R134. (Pubitemid 30146770)
4. Ardley H. C., Tan N. G., Rose S. A., Markham A. F., and, Robinson P. A., (2001) Features of the parkin/ariadne-like ubiquitin ligase, HHARI that regulate its interaction with the ubiquitin-conjugating enzyme, Ubch7. J. Biol. Chem. 276, 19640-19647.
5. Ardley H. C., Scott G. B., Rose S. A., Tan N. G., Markham A. F., and, Robinson P. A., (2003) Inhibition of proteasomal activity causes inclusion formation in neuronal and non neuronal cells overexpressing Parkin. Mol. Biol. Cell 14, 4541-4556. (Pubitemid 37444654)
6. Babu J. R., Geetha T., and, Wooten M. W., (2005) Sequestosome 1/p62 shuttles polyubiquitinated Tau for proteasomal degradation. J. Neurochem. 94, 192-203. (Pubitemid 40911405)
7. Ballatore C., Lee V. M., and, Trojanowski J. Q., (2007) Tau-mediated neurodegeneration in Alzheimer's disease and related disorders. Nat. Rev. Neurosci. 8, 663-672. (Pubitemid 47283144)
8. Ballinger C. A., Connell P., Wu Y., Hu Z., Thompson L. J., Yin L. Y., and, Patterson C., (1999) Identification of CHIP, a novel tetratricopeptide repeat-containing protein that interacts with heat shock proteins and negatively regulates chaperone functions. Mol. Cell. Biol. 19, 4535-4545. (Pubitemid 29242026)
9. Beer-Romero P., Glass S., and, Rolfe M., (1997) Antisense targeting of E6AP elevates p53 inn HPV-infected cells but not in normal cells. Oncogene 14, 595-602. (Pubitemid 27090962)
10. Behl C., Davis J. B., Lesley R., and, Schubert D., (1994) Hydrogen peroxide mediates amyloid beta protein toxicity. Cell 77, 817-827. (Pubitemid 24187680)
11. Bellomaria A., Barbato G., Melino G., Paci M., and, Melino S., (2010) Recognition of p63 by the E3 ligase ITCH: Effect of an ectodermal dysplasia mutant. Cell Cycle 9, 3730-3739.
12. Bennett E. J., Shaler T. A., Woodman B., Ryu K. Y., Zaitseva T. S., Becker C. H., Bates G. P., Schulman H., and, Kopito R. R., (2007) Global changes to the ubiquitin system in Huntington's disease. Nature 448, 704-708. (Pubitemid 47236860)
13. Bocharova N. A., Sokolov S. S., Knorre D. A., Skulachev V. P., and, Severin F. F., (2008) Unexpected link between anaphase promoting complex and the toxicity of expanded polyglutamines expressed in yeast. Cell Cycle 7, 3943-3946.
14. Bouman L., Schlierf A., Lutz A. K., et al. (2011) Parkin is transcriptionally regulated by ATF4: evidence for an interconnection between mitochondrial stress and ER stress. Cell Death Differ. 18, 769-782.
15. Branco J., Al-Ramahi I., Ukani L., Pérez A. M., Fernandez-Funez P., Rincõn-Limas D., and, Botas J., (2008) Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum. Mol. Genet. 17, 376-390.
16. BreTaud S., Allen C., Ingham P. W., and, Bandmann O., (2007) p53-dependent neuronal cell death in a DJ-1-deficient zebrafish model of Parkinson's disease. J. Neurochem. 100, 1626-1635. (Pubitemid 46376201)
17. Bulone D., Masino L., Thomas D. J., San Biagio P. L., and, Pastore A., (2006) The interplay between PolyQ and protein context delays aggregation by forming a reservoir of protofibrils. PLoS ONE 1, e111.
18. Burack M. A., Hartlein J., Flores H. P., Taylor-Reinwald L., Perlmutter J. S., and, Cairns N. J., (2010) In vivo amyloid imaging in autopsy-confirmed Parkinson disease with dementia. Neurology 74, 77-84.
19. Buratti E., and, Baralle F. E., (2009) The molecular links between TDP-43 dysfunction and neurodegeneration. Adv. Genet. 66, 1-34.
20. Burns M. P., Zhang L., Rebeck G. W., Querfurth H. W., and, Moussa C. E., (2009) Parkin promotes intracellular Abeta1-42 clearance. Hum. Mol. Genet. 18, 3206-3216.
21. Butcher J., (2005) Parkin gene therapy could treat Parkinson's disease. Lancet Neurol. 4, 82.
22. Carson R., McKnight A. J., Todd S., Liu W. W., Heggarty S., Craig D., McGuinness B., Irvine G. B., Passmore A. P., and, Johnston J. A., (2009) Variation in RTN3 and PPIL2 genes does not influence platelet membrane beta-secretase activity or susceptibility to Alzheimer's disease in the northern Irish population. Neuromol. Med. 11, 337-344.
23. Chafekar S. M., Zwart R., Veerhuis R., Vanderstichele H., Baas F., and, Scheper W., (2008) Increased Abeta1-42 production sensitizes neuroblastoma cells for ER stress toxicity. Curr. Alzheimer Res. 5, 469-474.
24. Chan N. C., Salazar A. M., Pham A. H., Sweredoski M. J., Kolawa N. J., Graham R. L., Hess S., and, Chan D. C., (2011) Broad activation of the ubiquitin-proteasome system by Parkin is critical for mitophagy. Hum. Mol. Genet. 20, 1726-1737.
25. Chapman A. P., Courtney S. C., Smith S. J., Rider C. C., and, Beesley P. W., (1992) Ubiquitin immunoreactivity of multiple polypeptides in rat brain synaptic membranes. Biochem. Soc. Trans. 20, 155S.
26. Chapman A. P., Smith S. J., Rider C. C., and, Beesley P. W., (1994) Multiple ubiquitin conjugates are present in rat brain synaptic membranes and postsynaptic densities. Neurosci. Lett. 168, 238-242. (Pubitemid 24097366)
27. Cheroni C., Marino M., Tortarolo M., Veglianese P., De Biasi S., Fontana E., Zuccarello L. V., Maynard C. J., Dantuma N. P., and, Bendotti C., (2009) Functional alterations of the ubiquitin-proteasome system in motor neurons of a mouse model of familial amyotrophic lateral sclerosis. Hum. Mol. Genet. 18, 82-96.
28. Chin L. S., Olzmann J. A., and, Li L., (2010) Parkin-mediated ubiquitin signalling in aggresome formation and autophagy. Biochem. Soc. Trans. 38, 144-149.
29. Choi J. S., Cho S., Park S. G., Park B. C., and, Lee D. H., (2004) Co-chaperone CHIP associates with mutant Cu/Zn-superoxide dismutase proteins linked to familial amyotrophic lateral sclerosis and promotes their degradation by proteasomes. Biochem. Biophys. Res. Commun. 321, 574-583. (Pubitemid 39127405)
30. Choi J. Y., Ryu J. H., Kim H. S., Park S. G., Bae K. H., Kang S., Myung P. K., Cho S., Park B. C., and, Lee do H., (2007) Co-chaperone CHIP promotes aggregation of ataxin-1. Mol. Cell Neurosci. 34, 69-79. (Pubitemid 46026931)
31. Chung K. K., and, Dawson T. M., (2004) Parkin and Hsp70 sacked by BAG5. Neuron 44, 899-901. (Pubitemid 39643083)
32. Chung K. K., Zhang Y., Lim K. L., Tanaka Y., Huang H., Gao J., Ross C. A., Dawson V. L., and, Dawson T. M., (2001) Parkin ubiquitinates the alpha-synuclein-interacting protein, synphilin-1: implications for Lewy-body formation in Parkinson disease. Nat. Med. 7, 1144-1150. (Pubitemid 33010024)
33. Chung K. K., Thomas B., Li X., Pletnikova O., Troncoso J. C., Marsh L., Dawson V. L., and, Dawson T. M., (2004) S-Nitrosylation of parkin regulates ubiquitination and compromises parkin's protective function. Science 304, 1328-1331. (Pubitemid 38697430)
34. Ciechanover A., (1998) The ubiquitin-proteasome pathway: on protein death and cell life. EMBO J. 17, 7151-7160. (Pubitemid 29002684)
35. Ciechanover A., (2005) Proteolysis: from the lysosome to ubiquitin and the proteasome. Nat. Rev. Mol. Cell Biol. 6, 79-87.
36. Ciechanover A., and, Brundin P., (2003) The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg. Neuron 40, 427-446. (Pubitemid 37244106)
37. Cleveland D. W., and, Rothstein J. D., (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat. Rev. Neurosci. 2, 806-819. (Pubitemid 33674062)
38. Colledge M., Snyder E. M., Crozier R. A., Soderling J. A., Jin Y., Langeberg L. K., Lu H., Bear M. F., and, Scott J. D., (2003) Ubiquitination regulates PSD-95 degradation and AMPA receptor surface expression. Neuron 40, 595-607. (Pubitemid 37494707)
39. Connell P., Ballinger C. A., Jiang J., Wu Y., Thompson L. J., Höhfeld J., and, Patterson C., (2001) The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins. Nat. Cell Biol. 3, 93-96. (Pubitemid 32114838)
40. Cookson M. R., and, Bandmann O., (2010) Parkinson's disease: insights from pathways. Hum. Mol. Genet. 19, R21-R27.
41. da Costa C. A., Sunyach C., Giaime E., et al. (2009) Transcriptional repression of p53 by Parkin and impairment by mutations associated with autosomal recessive juvenile Parkinson's disease. Nat. Cell Biol. 11, 1370-1375.
42. Dai Q., Zhang C., Wu Y., et al. (2003) CHIP activates HSF1 and confers protection against apoptosis and cellular stress. EMBO J. 22, 5446-5458. (Pubitemid 37279954)
43. Dai Q., Qian S. B., Li H. H., et al. (2005) Regulation of the cytoplasmic quality control protein degradation pathway by BAG2. J. Biol. Chem. 280, 38673-38681. (Pubitemid 43853770)
44. Darios F., Corti O., Lücking C. B., et al. (2003) Parkin prevents mitochondrial swelling and cytochrome c release in mitochondria-dependent cell death. Hum. Mol. Genet. 12, 517-526. (Pubitemid 36305590)
45. Dawson T. M., and, Dawson V. L., (2003) Molecular pathways of neurodegeneration in Parkinson's disease. Science 302, 819-822. (Pubitemid 37339614)
46. Deshaies R. J., (1999) SCF and Cullin/Ring H2-based ubiquitin ligases. Annu. Rev. Cell Dev. Biol. 15, 435-467. (Pubitemid 32250383)
47. Di Noto L., Whitson L. J., Cao X., Hart P. J., and, Levine R. L., (2005) Proteasomal degradation of mutant superoxide dismutases linked to amyotrophic lateral sclerosis. J. Biol. Chem. 280, 39907-39913. (Pubitemid 41779125)
48. Dickey C. A., Yue M., Lin W. L., et al. (2006) Deletion of the ubiquitin ligase CHIP leads to the accumulation, but not the aggregation, of both endogenous phospho- and caspase-3-cleaved Tau species. J. Neurosci. 26, 6985-6996. (Pubitemid 44309866)
49. Dickey C. A., Kamal A., Lundgren K., et al. (2007a) The high-affinity HSP90-CHIP complex recognizes and selectively degrades phosphorylated Tau client proteins. J. Clin. Invest. 117, 648-658. (Pubitemid 46348523)
50. Dickey C. A., Patterson C., Dickson D., and, Petrucelli L., (2007b) Brain CHIP: removing the culprits in neurodegenerative disease. Trends Mol. Med. 13, 32-38. (Pubitemid 46027726)
51. Dickey C. A., Koren J., Zhang Y. J., et al. (2008) Akt and CHIP coregulate Tau degradation through coordinated interactions. Proc. Natl. Acad. Sci. USA 105, 3622-3627. (Pubitemid 351723602)
52. Ding Q., and, Keller J. N., (2001) Proteasome inhibition in oxidative stress neurotoxicity: implications for heat shock proteins. J. Neurochem. 77, 1010-1017. (Pubitemid 32467056)
53. Dou F., Netzer W. J., Tanemura K., Li F., Hartl F. U., Takashima A., Gouras G. K., Greengard P., and, Xu H., (2003) Chaperones increase association of Tau protein with microtubules. Proc. Natl. Acad. Sci. USA 100, 721-726. (Pubitemid 36126119)
54. Duda J. E., Giasson B. I., Mabon M. E., Miller D. C., Golbe L. I., Lee V. M., and, Trojanowski J. Q., (2002) Concurrence of alpha-synuclein and Tau brain pathology in the Contursi kindred. Acta Neuropathol. 104, 7-11. (Pubitemid 36075353)
55. Durcan T. M., Kontogiannea M., Thorarinsdottir T., Fallon L., Williams A. J., Djarmati A., Fantaneanu T., Paulson H. L., and, Fon E. A., (2011) The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. Hum. Mol. Genet. 20, 141-154.
56. Espeseth A. S., Huang Q., Gates A., et al. (2006) A genome wide analysis of ubiquitin ligases in APP processing identifies a novel regulator of BACE1 mRNA levels. Mol. Cell. Neurosci. 33, 227-235. (Pubitemid 44692488)
57. Esposito L., Gan L., Yu G. Q., Essrich C., and, Mucke L., (2004) Intracellularly generated amyloid-beta peptide counteracts the antiapoptotic function of its precursor protein and primes proapoptotic pathways for activation by other insults in neuroblastoma cells. J. Neurochem. 91, 1260-1274. (Pubitemid 39658553)
58. Esser C., Scheffner M., and, Höhfeld J., (2005) The chaperone-associated ubiquitin ligase CHIP is able to target p53 for proteasomal degradation. J. Biol. Chem. 280, 27443-27448. (Pubitemid 41040787)
59. Favit A., Grimaldi M., and, Alkon D. L., (2000) Prevention of beta-amyloid neurotoxicity by blockade of the ubiquitin-proteasome proteolytic pathway. J. Neurochem. 75, 1258-1263. (Pubitemid 30660516)
60. Ferrari R., Kapogiannis D., Huey E. D., and, Momeni P., (2011) FTD and ALS: A Tale of Two Diseases. Curr. Alzheimer Res. 8, 273-294.
61. Fishman-Jacob T., Reznichenko L., Youdim M. B., and, Mandel S. A., (2009) A sporadic Parkinson disease model via silencing of the ubiquitin-proteasome/E3 ligase component SKP1A. J. Biol. Chem. 284, 32835-32845.
62. Fleischer A., Ghadiri A., Dessauge F., Duhamel M., Rebollo M. P., Alvarez-Franco F., and, Rebollo A., (2006) Modulating apoptosis as a target for effective therapy. Mol. Immunol. 43, 1065-1079. (Pubitemid 43063161)
63. Furukawa Y., Vigouroux S., Wong H., Guttman M., Rajput A. H., Ang L., Briand M., Kish S. J., and, Briand Y., (2002) Brain proteasomal function in sporadic Parkinson's disease and related disorders. Ann. Neurol. 51, 779-782. (Pubitemid 34568860)
64. Ganay T., Boizot A., Burrer R., Chauvin J. P., and, Bomont P., (2011) Sensory-motor deficits and neurofilament disorganization in gigaxonin-null mice. Mol. Neurodegener. 6, 25.
65. Gatchel J. R., and, Zoghbi H. Y., (2005) Diseases of unstable repeat expansion: mechanisms and common principles. Nat. Rev. Genet. 6, 743-755. (Pubitemid 41400832)
66. Ghribi O., Herman M. M., and, Savory J., (2003) Lithium inhibits Abeta-induced stress in endoplasmic reticulum of rabbit hippocampus but does not prevent oxidative damage and Tau phosphorylation. J. Neurosci. Res. 71, 853-862. (Pubitemid 36293006)
67. Glickman M. H., and, Ciechanover A., (2002) The ubiquitin-proteasome proteolytic pathway: destruction for the sake of construction. Physiol. Rev. 82, 373-428. (Pubitemid 34654457)
68. Godin J. D., Poizat G., Hickey M. A., Maschat F., and, Humbert S., (2010) Mutant huntingtin-impaired degradation of beta-catenin causes neurotoxicity in Huntington's disease. EMBO J. 29, 2433-2445.
69. Goedert M., (2001) Alpha-synuclein and neurodegenerative diseases. Nat. Rev. Neurosci. 2, 492-501. (Pubitemid 33676584)
70. Goldberg M. S., Fleming S. M., Palacino J. J., et al. (2003) Parkin-deficient mice exhibit nigrostriatal deficits but not loss of dopaminergic neurons. J. Biol. Chem. 278, 43628-43635. (Pubitemid 37345989)
71. Gomperts S. N., Rentz D. M., Moran E., et al. (2008) Imaging amyloid deposition in Lewy body diseases. Neurology 71, 903-910.
72. Gong B., Chen F., Pan Y., Arrieta-Cruz I., Yoshida Y., Haroutunian V., and, Pasinetti G. M., (2010) SCFFbx2-E3-ligase-mediated degradation of BACE1 attenuates Alzheimer's disease amyloidosis and improves synaptic function. Aging Cell 9, 1018-1031.
73. Goryunov D., and, Liem R. K., (2007) CHIP-ping away at Tau. J. Clin. Invest. 117, 590-592. (Pubitemid 46348514)
74. Götz J., Chen F., van Dorpe J., and, Nitsch R. M., (2001) Formation of neurofibrillary tangles in P301l Tau transgenic mice induced by Abeta 42 fibrils. Science 293, 1491-1495. (Pubitemid 32807681)
75. Greene J. C., Whitworth A. J., Kuo I., Andrews L. A., Feany M. B., and, Pallanck L. J., (2003) Mitochondrial pathology and apoptotic muscle degeneration in Drosophila parkin mutants. Proc. Natl. Acad. Sci. USA 100, 4078-4083. (Pubitemid 36418160)
76. Greene J. C., Whitworth A. J., Andrews L. A., Parker T. J., and, Pallanck L. J., (2005) Genetic and genomic studies of Drosophila parkin mutants implicate oxidative stress and innate immune responses in pathogenesis. Hum. Mol. Genet. 14, 799-811. (Pubitemid 40403276)
77. Gregori L., Fuchs C., Figueiredo-Pereira M. E., Van Nostrand W. E., and, Goldgaber D., (1995) Amyloid beta-protein inhibits ubiquitin-dependent protein degradation in vitro. J. Biol. Chem. 270, 19702-19708.
78. Guerrero R., Navarro P., Gallego E., Avila J., de Yebenes J. G., and, Sanchez M. P., (2008) Park2-null/Tau transgenic mice reveal a functional relationship between parkin and Tau. J. Alzheimers Dis. 13, 161-172. (Pubitemid 352038522)
79. Haass C., and, Selkoe D. J., (2007) Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide. Nat. Rev. Mol. Cell Biol. 8, 101-112. (Pubitemid 46432529)
80. Hamilton R. L., (2000) Lewy bodies in Alzheimer's disease: a neuropathological review of 145 cases using alpha-synuclein immunohistochemistry. Brain Pathol. 10, 378-384. (Pubitemid 30431290)
81. Harrington C. R., Perry R. H., Perry E. K., Hurt J., McKeith I. G., Roth M., and, Wischik C. M., (1994) Senile dementia of Lewy body type and Alzheimer type are biochemically distinct in terms of paired helical filaments and hyperphosphorylated Tau protein. Dementia 5, 215-228. (Pubitemid 24231097)
82. Hatakeyama S., Yada M., Matsumoto M., Ishida N., and, Nakayama K. I., (2001) U box proteins as a new family of ubiquitin-protein ligases. J. Biol. Chem. 276, 33111-33120.
83. Hatakeyama S., Matsumoto M., Kamura T., Murayama M., Chui D. H., Planel E., Takahashi R., Nakayama K. I., and, Takashima A., (2004) U-box protein carboxyl terminus of Hsc70-interacting protein (CHIP) mediates poly-ubiquitylation preferentially on four-repeat Tau and is involved in neurodegeneration of Tauopathy. J. Neurochem. 91, 299-307. (Pubitemid 39363429)
84. Hegde A. N., (2004) Ubiquitin-proteasome-mediated local protein degradation and synaptic plasticity. Prog. Neurobiol. 73, 311-357. (Pubitemid 39093810)
85. Hegde A. N., and, Upadhya S. C., (2011) Role of ubiquitin-proteasome- mediated proteolysis in nervous system disease. Biochim. Biophys. Acta 1809, 128-140.
86. Hermey G., (2011) Targeting amyloid precursor protein. Ann. Neurol. 69, 8-10.
87. Hershko A., and, Ciechanover A., (1998) The ubiquitin system. Annu. Rev. Biochem. 67, 425-479. (Pubitemid 28411135)
88. Hershko A., Ciechanover A., and, Varshavsky A., (2000) Basic Medical Research Award. The ubiquitin system. Nat. Med. 6, 1073-1081.
89. Hoshino T., Nakaya T., Araki W., Suzuki K., Suzuki T., and, Mizushima T., (2007) Endoplasmic reticulum chaperones inhibit the production of amyloid-beta peptides. Biochem. J. 402, 581-589. (Pubitemid 46423895)
90. Hsieh H., Boehm J., Sato C., Iwatsubo T., Tomita T., Sisodia S., and, Malinow R., (2006) AMPAR removal underlies Abeta-induced synaptic depression and dendritic spine loss. Neuron 52, 831-843. (Pubitemid 44828454)
91. Huynh D. P., Scoles D. R., Ho T. H., Del Bigio M. R., and, Pulst S. M., (2000) Parkin is associated with actin filaments in neuronal and nonneural cells. Ann. Neurol. 48, 737-744.
92. Hwang J. R., Zhang C., and, Patterson C., (2005) C-terminus of heat shock protein 70-interacting protein facilitates degradation of apoptosis signal-regulating kinase 1 and inhibits apoptosis signal-regulating kinase 1-dependent apoptosis. Cell Stress Chaperones 10, 147-156. (Pubitemid 40942475)
93. Hyun D. H., Lee M., Hattori N., Kubo S., Mizuno Y., Halliwell B., and, Jenner P., (2002) Effect of wild-type or mutant Parkin on oxidative damage, nitric oxide, antioxidant defenses, and the proteasome. J. Biol. Chem. 277, 28572-28577. (Pubitemid 41079284)
94. Hyun D. H., Lee M., Halliwell B., and, Jenner P., (2003) Proteasomal inhibition causes the formation of protein aggregates containing a wide range of proteins, including nitrated proteins. J. Neurochem. 86, 363-373. (Pubitemid 36842435)
95. Ihara Y., (2006) Neurofibrillary tangles/paired helical filaments (1981-83). J. Alzheimers Dis. 9, 209-217. (Pubitemid 44253314)
96. Illuzzi J. L., Vickers C. A., and, Kmiec E. B., (2011) Modifications of p53 and the DNA Damage Response in Cells Expressing Mutant Form of the Protein Huntingtin. J. Mol. Neurosci. 45, 256-268.
97. Imai Y., Soda M., and, Takahashi R., (2000) Parkin suppresses unfolded protein stress-induced cell death through its E3 ubiquitin-protein ligase activity. J. Biol. Chem. 275, 35661-35664.
98. Imai Y., Soda M., Inoue H., Hattori N., Mizuno Y., and, Takahashi R., (2001) An unfolded putative transmembrane polypeptide, which can lead to endoplasmic reticulum stress, is a substrate of Parkin. Cell 105, 891-902. (Pubitemid 32635090)
99. Imai Y., Soda M., Hatakeyama S., Akagi T., Hashikawa T., Nakayama K. I., and, Takahashi R., (2002) CHIP is associated with Parkin, a gene responsible for familial Parkinson's disease, and enhances its ubiquitin ligase activity. Mol. Cell 10, 55-67. (Pubitemid 34876562)
100. Imarisio S., Carmichael J., Korolchuk V., et al. (2008) Huntington's disease: from pathology and genetics to potential therapies. Biochem. J. 412, 191-209. (Pubitemid 351758226)
101. Ishigaki S., Hishikawa N., Niwa J., Iemura S., Natsume T., Hori S., Kakizuka A., Tanaka K., and, Sobue G., (2004) Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders. J. Biol. Chem. 279, 51376-51385. (Pubitemid 40017885)
102. Ittner L. M., and, Götz J., (2011) Amyloid-β and Tau-a toxic pas de deux in Alzheimer's disease. Nat. Rev. Neurosci. 12, 65-72.
103. Jackson P. K., Eldridge A. G., Freed E., Furstenthal L., Hsu J. Y., Kaiser B. K., and, Reimann J. D., (2000) The lore of the RINGs: substrate recognition and catalysis by ubiquitin ligases. Trends Cell Biol. 10, 429-439.
104. Jana N. R., Dikshit P., Goswami A., Kotliarova S., Murata S., Tanaka K., and, Nukina N., (2005) Co-chaperone CHIP associates with expanded polyglutamine protein and promotes their degradation by proteasomes. J. Biol. Chem. 280, 11635-11640. (Pubitemid 40418477)
105. Janssens J., Kleinberger G., Wils H., and, Van Broeckhoven C., (2011) The role of mutant TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Biochem. Soc. Trans. 39, 954-959.
106. Jiang J., Ballinger C. A., Wu Y., Dai Q., Cyr D. M., Höhfeld J., and, Patterson C., (2001) CHIP is a U-box-dependent E3 ubiquitin ligase: identification of Hsc70 as a target for ubiquitylation. J. Biol. Chem. 276, 42938-42944.
107. Joazeiro C. A., and, Weissman A. M., (2000) RING finger proteins: mediators of ubiquitin ligase activity. Cell 102, 549-552.
108. Julien J. P., (2001) Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded. Cell 104, 581-591.
109. Kadowaki H., Nishitoh H., Urano F., et al. (2005) Amyloid beta induces neuronal cell death through ROS-mediated ASK1 activation. Cell Death Differ. 12, 19-24. (Pubitemid 40090025)
110. Kampinga H. H., Kanon B., Salomons F. A., Kabakov A. E., and, Patterson C., (2003) Overexpression of the cochaperone CHIP enhances Hsp70-dependent folding activity in mammalian cells. Mol. Cell. Biol. 23, 4948-4958. (Pubitemid 36793340)
111. Kaneko H., Kakita A., Kasuga K., et al. (2007) Enhanced accumulation of phosphorylated alpha-synuclein and elevated beta-amyloid 42/40 ratio caused by expression of the presenilin-1 deltaT440 mutant associated with familial Lewy body disease and variant Alzheimer's disease. J. Neurosci. 27, 13092-13097. (Pubitemid 350225495)
112. Kaneko M., Koike H., Saito R., Kitamura Y., Okuma Y., and, Nomura Y., (2010) Loss of HRD1-mediated protein degradation causes amyloid precursor protein accumulation and amyloid-beta generation. J. Neurosci. 30, 3924-3932.
113. Katsanis N., and, Fisher E. M., (1998) Identification, expression, and chromosomal localization of ubiquitin conjugating enzyme 7 (UBE2G2), a human homologue of the Saccharomyces cerevisiae ubc7 gene. Genomics 51, 128-131. (Pubitemid 28373381)
114. Keller J. N., Hanni K. B., and, Markesbery W. R., (2000a) Impaired proteasome function in Alzheimer's disease. J. Neurochem. 75, 436-439. (Pubitemid 30418211)
115. Keller J. N., Lauderback C. M., Butterfield D. A., Kindy M. S., Yu J., and, Markesbery W. R., (2000b) Amyloid beta-peptide effects on synaptosomes from apolipoprotein E-deficient mice. J. Neurochem. 74, 1579-1586. (Pubitemid 30163979)
116. Khandelwal P. J., and, Moussa C. E., (2010) The relationship between parkin and protein aggregation in neurodegenerative diseases. Front. Psychiatry 1, 15.
117. Khandelwal P. J., Herman A. M., Hoe H. S., Rebeck G. W., and, Moussa C. E., (2011) Parkin mediates beclin-dependent autophagic clearance of defective mitochondria and ubiquitinated Abeta in AD models. Hum. Mol. Genet. 20, 2091-2102.
118. Kiernan M. C., Vucic S., Cheah B. C., Turner M. R., Eisen A., Hardiman O., Burrell J. R., and, Zoing M. C., (2011) Amyotrophic lateral sclerosis. Lancet 377, 942-955.
119. Kim C., Srivastava S., Rice M., Godenschwege T. A., Bentley B., Ravi S., Shao S., Woodard C. T., and, Schwartz L. M., (2011) Expression of human amyloid precursor protein in the skeletal muscles of Drosophila results in age- and activity-dependent muscle weakness. BMC Physiol. 11, 7.
120. Kimura M., Hattori T., Matsuda Y., Yoshioka T., Sumi N., Umeda Y., Nakashima S., and, Okano Y., (1997) cDNA cloning, characterization, and chromosome mapping of UBE2E2 encoding a human ubiquitin-conjugating E2 enzyme. Cytogenet. Cell Genet. 78, 107-111. (Pubitemid 27480759)
121. Kirik D., and, Björklund A., (2005) Parkinson's disease: viral vector delivery of parkin generates model results in rats. Gene Ther. 12, 727-729. (Pubitemid 40668410)
122. Kitada T., Asakawa S., Hattori N., Matsumine H., Yamamura Y., Minoshima S., Yokochi M., Mizuno Y., and, Shimizu N., (1998) Mutations in the parkin gene cause autosomal recessive juvenile Parkinsonism. Nature 392, 605-608. (Pubitemid 28207717)
123. Klein C., and, Schlossmacher M. G., (2007) Parkinson disease, 10 years after its genetic revolution: multiple clues to a complex disorder. Neurology 69, 2093-2104. (Pubitemid 350175712)
124. Klein R. L., Dayton R. D., Henderson K. M., and, Petrucelli L., (2006) Parkin is protective for substantia nigra dopamine neurons in a Tau gene transfer neurodegeneration model. Neurosci. Lett. 401 (1-2), 130-135.
125. Ko H. S., Lee Y., Shin J. H., Karuppagounder S. S., Gadad B. S., Koleske A. J., Pletnikova O., Troncoso J. C., Dawson V. L., and, Dawson T. M., (2010) Phosphorylation by the c-Abl protein tyrosine kinase inhibits parkin's ubiquitination and protective function. Proc. Natl. Acad. Sci. USA 107, 16691-16696.
126. Koegl M., Hoppe T., Schlenker S., Ulrich H. D., Mayer T. U., and, Jentsch S., (1999) A novel ubiquitination factor, E4, is involved in multiubiquitin chain assembly. Cell 96, 635-644. (Pubitemid 29122806)
127. Koo E. H., Squazzo S. L., Selkoe D. J., and, Koo C. H., (1996) Trafficking of cell-surface amyloid beta-protein precursor. I. Secretion, endocytosis and recycling as detected by labeled monoclonal antibody. J. Cell Sci. 109, 991-998.
128. Kosik K. S., and, Shimura H., (2005) Phosphorylated Tau and the neurodegenerative foldopathies. Biochim. Biophys. Acta 1739, 298-310. (Pubitemid 40018546)
129. Kotzbauer P. T., Trojanowsk J. Q., and, Lee V. M., (2001) Lewy body pathology in Alzheimer's disease. J. Mol. Neurosci. 17, 225-232. (Pubitemid 33063526)
130. Kumar P., Ambasta R. K., Veereshwarayya V., Rosen K. M., Kosik K. S., Band H., Mestril R., Patterson C., and, Querfurth H. W., (2007) CHIP and HSPs interact with beta-APP in a proteasome-dependent manner and influence Abeta metabolism. Hum. Mol. Genet. 16, 848-864. (Pubitemid 47061630)
131. Kuroda Y., Mitsui T., Kunishige M., Shono M., Akaike M., Azuma H., and, Matsumoto T., (2006) Parkin enhances mitochondrial biogenesis in proliferating cells. Hum. Mol. Genet. 15, 883-895. (Pubitemid 43338230)
132. Lagier-Tourenne C., Polymenidou M., and, Cleveland D. W., (2010) TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Hum. Mol. Genet. 19, R46-R64.
133. Lashley T., Holton J. L., Gray E., Kirkham K., O'Sullivan S. S., Hilbig A., Wood N. W., Lees A. J., and, Revesz T., (2008) Cortical alpha-synuclein load is associated with amyloid-beta plaque burden in a subset of Parkinson's disease patients. Acta Neuropathol. 115, 417-425.
134. Ledesma M. D., Galvan C., Hellias B., Dotti C., and, Jensen P. H., (2002) Astrocytic but not neuronal increased expression and redistribution of parkin during unfolded protein stress. J. Neurochem. 83, 1431-1440. (Pubitemid 35477482)
135. Lee J. T., Wheeler T. C., Li L., and, Chin L. S., (2008) Ubiquitination of alpha-synuclein by Siah-1 promotes alpha-synuclein aggregation and apoptotic cell death. Hum. Mol. Genet. 17, 906-917. (Pubitemid 351359706)
136. Léger B., Vergani L., Sorarù G., Hespel P., Derave W., Gobelet C., D'Ascenzio C., Angelini C., and, Russell A. P., (2006) Human skeletal muscle atrophy in amyotrophic lateral sclerosis reveals a reduction in Akt and an increase in atrogin-1. FASEB J. 20, 583-585. (Pubitemid 46671257)
137. Leissring M. A., Farris W., Chang A. Y., Walsh D. M., Wu X., Sun X., Frosch M. P., and, Selkoe D. J., (2003) Enhanced proteolysis of beta-amyloid in APP transgenic mice prevents plaque formation, secondary pathology, and premature death. Neuron 40, 1087-1093. (Pubitemid 38032786)
138. Li S., and, Li X. J., (2006) Multiple pathways contribute to the pathogenesis of Huntington disease. Mol. Neurodegener. 1, 19.
139. Li X. J., and, Li S., (2011) Proteasomal dysfunction in aging and Huntington disease. Neurobiol. Dis. 43, 4-8.
140. Li J., Pauley A. M., Myers R. L., Shuang R., Brashler J. R., Yan R., Buhl A. E., Ruble C., and, Gurney M. E., (2002) SEL-10 interacts with presenilin 1, facilitates its ubiquitination, and alters A-beta peptide production. J. Neurochem. 82, 1540-1548.
141. Li L. B., Yu Z., Teng X., and, Bonini N. M., (2008) RNA toxicity is a component of ataxin-3 degeneration in Drosophila. Nature 453, 1107-1111. (Pubitemid 351871727)
142. Lim K. L., (2007) Ubiquitin-proteasome system dysfunction in Parkinson's disease: current evidence and controversies. Expert Rev. Proteomics 4, 769-781.
143. Liu Q. Y., Lei J. X., Sikorska M., and, Liu R., (2008) A novel brain-enriched E3 ubiquitin ligase RNF182 is up regulated in the brains of Alzheimer's patients and targets ATP6V0C for degradation. Mol. Neurodegener. 3, 4.
144. Lo Bianco C., Schneider B. L., Bauer M., Sajadi A., Brice A., Iwatsubo T., and, Aebischer P., (2004) Lentiviral vector delivery of parkin prevents
145. Lopez Salon M., Pasquini L., Besio Moreno M., Pasquini J. M., and, Soto E., (2003) Relationship between beta-amyloid degradation and the 26S proteasome in neural cells. Exp. Neurol. 180, 131-143. (Pubitemid 36398168)
146. Lõpez Salon M., Morelli L., Castaño E. M., Soto E. F., and, Pasquini J. M., (2000) Defective ubiquitination of cerebral proteins in Alzheimer's disease. J. Neurosci. Res. 62, 302-310.
147. Lücking C. B., Dürr A., Bonifati V., et al. (2000) Association between early-onset Parkinson's disease and mutations in the parkin gene. N. Engl. J. Med. 342, 1560-1567. (Pubitemid 30340501)
148. Luo W., Dou F., Rodina A., et al. (2007) Roles of heat-shock protein 90 in maintaining and facilitating the neurodegenerative phenotype in Tauopathies. Proc. Natl. Acad. Sci. USA 104, 9511-9516. (Pubitemid 47185810)
149. Madura K., Dohmen R. J., and, Varshavsky A., (1993) N-recognin/Ubc2 interactions in the N-end rule pathway. J. Biol. Chem. 268, 12046-12054. (Pubitemid 23168169)
150. Maestre C., Delgado-Esteban M., Gomez-Sanchez J. C., Bolaños J. P., and, Almeida A., (2008) Cdk5 phosphorylates Cdh1 and modulates cyclin B1 stability in excitotoxicity. EMBO J. 27, 2736-2745.
151. Manczak M., Calkins M. J., and, Reddy P. H., (2011) Impaired mitochondrial dynamics and abnormal interaction of amyloid beta with mitochondrial protein Drp1 in neurons from patients with Alzheimer's disease: implications for neuronal damage. Hum. Mol. Genet. 20, 2495-2509.
152. Manfredsson F. P., Burger C., Sullivan L. F., Muzyczka N., Lewin A. S., and, Mandel R. J., (2007) rAAVmediated nigral human parkin over-expression partially ameliorates motor deficits via enhanced dopamine neurotransmission in a rat model of Parkinson's disease. Exp. Neurol. 207, 289-301. (Pubitemid 47429661)
153. Mashimo T., Hadjebi O., Amair-Pinedo F., Tsurumi T., Langa F., Serikawa T., Sotelo C., Guénet J. L., and, Rosa J. L., (2009) Progressive Purkinje cell degeneration in tambaleante mutant mice is a consequence of a missense mutation in HERC1 E3 ubiquitin ligase. PLoS Genet. 5, e1000784.
154. Matsuda N., and, Tanaka K., (2010) Does impairment of the ubiquitin-proteasome system or the autophagy-lysosome pathway predispose individuals to neurodegenerative disorders such as Parkinson's disease? J. Alzheimers Dis. 19, 1-9.
155. Mattson M. P., and, Goodman Y., (1995) Different amyloidogenic peptides share a similar mechanism of neurotoxicity involving reactive oxygen species and calcium. Brain Res. 676, 219-224.
156. McClellan A. J., and, Frydman J., (2001) Molecular chaperones and the art of recognizing a lost cause. Nat. Cell Biol. 3, E51-E53. (Pubitemid 32118358)
157. McDonough H., and, Patterson C., (2003) CHIP: a link between the chaperone and proteasome systems. Cell Stress Chaperones 8, 303-308. (Pubitemid 38222876)
158. McNaught K. S., Olanow C. W., Halliwell B., Isacson O., and, Jenner P., (2001) Failure of the ubiquitin-proteasome system in Parkinson's disease. Nat. Rev. Neurosci. 2, 589-594. (Pubitemid 33679832)
159. Meacham G. C., Patterson C., Zhang W., Younger J. M., and, Cyr D. M., (2001) The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation. Nat. Cell Biol. 3, 100-105. (Pubitemid 32114840)
160. Mei J., and, Niu C., (2010) Alterations of Hrd1 expression in various encephalic regional neurons in 6-OHDA model of Parkinson's disease. Neurosci. Lett. 474, 63-68.
161. Menéndez J., Rodríguez-Navarro J. A., Solano R. M., Casarejos M. J., Rodal I., Guerrero R., Sánchez M. P., Avila J., Mena M. A., and, de Yébenes J. G., (2006) Suppression of Parkin enhances nigrostriatal and motor neuron lesion in mice over-expressing human-mutated Tau protein. Hum. Mol. Genet. 15, 2045-2058. (Pubitemid 43923395)
162. Miller V. M., Nelson R. F., Gouvion C. M., Williams A., Rodriguez-Lebron E., Harper S. Q., Davidson B. L., Rebagliati M. R., and, Paulson H. L., (2005) CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J. Neurosci. 25, 9152-9161. (Pubitemid 41500823)
163. Mo X., Liu D., Li W., et al. (2010) Genetic screening for mutations in the Nrdp1 gene in Parkinson disease patients in a Chinese population. Parkinsonism Relat. Disord. 16, 222-224.
164. Moore D. J., (2006) Parkin: a multifaceted ubiquitin ligase. Biochem. Soc. Trans. 34, 749-753. (Pubitemid 44796412)
165. Moore D. J., West A. B., Dawson V. L., and, Dawson T. M., (2005) Molecular pathophysiology of Parkinson's disease. Annu. Rev. Neurosci. 28, 57-87. (Pubitemid 41098899)
166. Moore D. J., West A. B., Dikeman D. A., Dawson V. L., and, Dawson T. M., (2008) Parkin mediates the degradation-independent ubiquitination of Hsp70. J. Neurochem. 105, 1806-1819. (Pubitemid 351678490)
167. Morett E., and, Bork P., (1999) A novel transactivation domain in parkin. Trends Biochem. Sci. 24, 229-231. (Pubitemid 29317288)
168. Mori F., Nishie M., Piao Y. S., Kito K., Kamitani T., Takahashi H., and, Wakabayashi K., (2005) Accumulation of NEDD8 in neuronal and glial inclusions of neurodegenerative disorders. Neuropathol. Appl. Neurobiol. 31, 53-61. (Pubitemid 40222825)
169. Morishima Y., Wang A. M., Yu Z., Pratt W. B., Osawa Y., and, Lieberman A. P., (2008) CHIP deletion reveals functional redundancy of E3 ligases in promoting degradation of both signaling proteins and expanded glutamine proteins. Hum. Mol. Genet. 17, 3942-3952. (Pubitemid 352762855)
170. Morishima-Kawashima M., Hasegawa M., Takio K., Suzuki M., Titani K., and, Ihara Y., (1993) Ubiquitin is conjugated with amino-terminally processed Tau in paired helical filaments. Neuron 10, 1151-1160. (Pubitemid 23194405)
171. Moussa C. E., (2009) Parkin attenuates wild-type Tau modification in the presence of beta-amyloid and alpha-synuclein. J. Mol. Neurosci. 37, 25-36.
172. Muchowski P. J., and, Wacker J. L., (2005) Modulation of neurodegeneration by molecular chaperones. Nat. Rev. Neurosci. 6, 11-22. (Pubitemid 40052135)
173. Murata S., Minami Y., Minami M., Chiba T., and, Tanaka K., (2001) CHIP is a chaperone-dependent E3 ligase that ubiquitylates unfolded protein. EMBO Rep. 2, 1133-1138. (Pubitemid 34055961)
174. Murata S., Chiba T., and, Tanaka K., (2003) CHIP: a quality-control E3 ligase collaborating with molecular chaperones. Int. J. Biochem. Cell Biol. 35, 572-578. (Pubitemid 36369506)
175. Murata S., Yashiroda H., and, Tanaka K., (2009) Molecular mechanisms of proteasome assembly. Nat. Rev. Mol. Cell Biol. 10, 104-115.
176. Nair V. D., McNaught K. S., González-Maeso J., Sealfon S. C., and, Olanow C. W., (2006) p53 mediates nontranscriptional cell death in dopaminergic cells in response to proteasome inhibition. J. Biol. Chem. 281, 39550-39560. (Pubitemid 46041915)
177. Nakamura T., Cieplak P., Cho D. H., Godzik A., and, Lipton S. A., (2010) S-nitrosylation of Drp1 links excessive mitochondrial fission to neuronal injury in neurodegeneration. Mitochondrion 10, 573-578.
178. Niwa J., Ishigaki S., Hishikawa N., Yamamoto M., Doyu M., Murata S., Tanaka K., Taniguchi N., and, Sobue G., (2002) Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity. J. Biol. Chem. 277, 36793-36798.
179. Novak M. J., and, Tabrizi S. J., (2010) Huntington's disease. Br. Med. J. 340, c3109.
180. Oddo S., Caccamo A., Tseng B., Cheng D., Vasilevko V., Cribbs D. H., and, LaFerla F. M., (2008) Blocking Abeta42 accumulation delays the onset and progression of Tau pathology via the C terminus of heat shock protein70-interacting protein: a mechanistic link between Abeta and Tau pathology. J. Neurosci. 28, 12163-12175.
181. Olzscha H., Schermann S. M., Woerner A. C., Pinkert S., Hecht M. H., Tartaglia G. G., Vendruscolo M., Hayer-Hartl M., Hartl F. U., and, Vabulas R. M., (2011) Amyloid-like aggregates sequester numerous metastable proteins with essential cellular functions. Cell 144, 67-78.
182. Orr H. T., and, Zoghbi H. Y., (2000) Reversing neurodegeneration: a promise unfolds. Cell 101, 1-4.
183. Palacino J. J., Sagi D., Goldberg M. S., Krauss S., Motz C., Wacker M., Klose J., and, Shen J., (2004) Mitochondrial dysfunction and oxidative damage in parkin-deficient mice. J. Biol. Chem. 279, 18614-18622. (Pubitemid 38623283)
184. Pandey U. B., Nie Z., Batlevi Y., et al. (2007) HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature 447, 859-863. (Pubitemid 46920134)
185. Park Y. Y., Lee S., Karbowski M., Neutzner A., Youle R. J., and, Cho H., (2010) Loss of MARCH5 mitochondrial E3 ubiquitin ligase induces cellular senescence through dynamin-related protein 1 and mitofusin 1. J. Cell Sci. 123, 619-626.
186. Perry G., Rizzuto N., Autilio-Gambetti L., and, Gambetti P., (1985) Paired helical filaments from Alzheimer disease patients contain cytoskeletal components. Proc. Natl. Acad. Sci. USA 82, 3916-3920. (Pubitemid 15246464)
187. Perry G., Friedman R., Shaw G., and, Chau V., (1987) Ubiquitin is detected in neurofibrillary tangles and senile plaque neurites of Alzheimer disease brains. Proc. Natl. Acad. Sci. USA 84, 3033-3036. (Pubitemid 17064772)
188. Perucho J., Casarejos M. J., Rubio I., et al. (2010) The effects of parkin suppression on the behaviour, amyloid processing, and cell survival in APP mutant transgenic mice. Exp. Neurol. 221, 54-67.
189. Petrucelli L., O'Farrell C., Lockhart P. J., et al. (2002) Parkin protects against the toxicity associated with mutant alpha-synuclein: proteasome dysfunction selectively affects catecholaminergic neurons. Neuron 36, 1007-1019. (Pubitemid 36044504)
190. Petrucelli L., Dickson D., Kehoe K., et al. (2004) CHIP and Hsp70 regulate Tau ubiquitination, degradation and aggregation. Hum. Mol. Genet. 13, 703-714. (Pubitemid 38455958)
191. Peviani M., Caron I., Pizzasegola C., Gensano F., Tortarolo M., and, Bendotti C., (2010) Unraveling the complexity of amyotrophic lateral sclerosis: recent advances from the transgenic mutant SOD1 mice. CNS Neurol. Disord. Drug Targets 9, 491-503.
192. Pickart C. M., and, Cohen R. E., (2004) Proteasomes and their kin: proteases in the machine age. Nat. Rev. Mol. Cell Biol. 5, 177-187. (Pubitemid 38325799)
193. Plant P. J., Correa J., Goldenberg N., Bain J., and, Batt J., (2009) The inositol phosphatase MTMR4 is a novel target of the ubiquitin ligase Nedd4. Biochem. J. 419, 57-63.
194. Proctor C. J., and, Gray D. A., (2010) GSK3 and p53-is there a link in Alzheimer's disease? Mol. Neurodegener. 5, 7.
195. Qian S. B., McDonough H., Boellmann F., Cyr D. M., and, Patterson C., (2006) CHIP-mediated stress recovery by sequential ubiquitination of substrates and Hsp70. Nature 440, 551-555.
196. Querfurth H. W., and, LaFerla F. M., (2010) Alzheimer's disease. N. Engl. J. Med. 362, 329-344.
197. Rangone H., Pardo R., Colin E., Girault J. A., Saudou F., and, Humbert S., (2005) Phosphorylation of arfaptin 2 at Ser260 by Akt Inhibits PolyQ-huntingtin-induced toxicity by rescuing proteasome impairment. J. Biol. Chem. 280, 22021-22028. (Pubitemid 40827856)
198. Reddy P. H., (2007) Mitochondrial dysfunction in aging and Alzheimer's disease: strategies to protect neurons. Antioxid. Redox Signal. 9, 1647-1658. (Pubitemid 47462968)
199. Riederer B. M., Leuba G., Vernay A., and, Riederer I. M., (2011) The role of the ubiquitin proteasome system in Alzheimer's disease. Exp. Biol. Med. (Maywood) 236, 268-276.
200. Roberson E. D., Scearce-Levie K., Palop J. J., Yan F., Cheng I. H., Wu T., Gerstein H., Yu G. Q., and, Mucke L., (2007) Reducing endogenous Tau ameliorates amyloid beta-induced deficits in an Alzheimer's disease mouse model. Science 316, 750-754. (Pubitemid 46717684)
201. Robertson A. L., and, Bottomley S. P., (2010) Towards the treatment of polyglutamine diseases: the modulatory role of protein context. Curr. Med. Chem. 17, 3058-3068.
202. Rodríguez-Navarro J. A., Gõmez A., Rodal I., et al. (2008) Parkin deletion causes cerebral and systemic amyloidosis in human mutated Tau over-expressing mice. Hum. Mol. Genet. 17, 3128-3143.
203. Rosen D. R., (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 364, 362. (Pubitemid 23265284)
204. Rosen K. M., Veereshwarayya V., Moussa C. E., Fu Q., Goldberg M. S., Schlossmacher M. G., Shen J., and, Querfurth H. W., (2006) Parkin protects against mitochondrial toxins and beta-amyloid accumulation in skeletal muscle cells. J. Biol. Chem. 281 (18), 12809-12816. (Pubitemid 43855373)
205. Rosen K. M., Moussa C. E., Lee H. K., Kumar P., Kitada T., Qin G., Fu Q., and, Querfurth H. W., (2010) Parkin reverses intracellular beta-amyloid accumulation and its negative effects on proteasome function. J. Neurosci. Res. 88, 167-178.
206. Rosser M. F., Washburn E., Muchowski P. J., Patterson C., and, Cyr D. M., (2007) Chaperone functions of the E3 ubiquitin ligase CHIP. J. Biol. Chem. 282, 22267-22277. (Pubitemid 47267320)
207. von Rotz R. C., Kins S., Hipfel R., von der Kammer H., and, Nitsch R. M., (2005) The novel cytosolic RING finger protein dactylidin is up-regulated in brains of patients with Alzheimer's disease. Eur. J. Neurosci. 21, 1289-1298. (Pubitemid 40516627)
208. Sahara N., Murayama M., Mizoroki T., Urushitani M., Imai Y., Takahashi R., Murata S., Tanaka K., and, Takashima A., (2005) In vivo evidence of CHIP up-regulation attenuating Tau aggregation. J. Neurochem. 94, 1254-1263. (Pubitemid 41232581)
209. Sahara N., Maeda S., Yoshiike Y., Mizoroki T., Yamashita S., Murayama M., Park J. M., Saito Y., Murayama S., and, Takashima A., (2007) Molecular chaperone-mediated Tau protein metabolism counteracts the formation of granular Tau oligomers in human brain. J. Neurosci. Res. 85, 3098-3108. (Pubitemid 350057365)
210. Saito R., Kaneko M., Okuma Y., and, Nomura Y., (2010) Correlation between decrease in protein levels of ubiquitin ligase HRD1 and amyloid-beta production. J. Pharmacol. Sci. 113, 285-288.
211. Sakahira H., Breuer P., Hayer-Hartl M. K., and, Hartl F. U., (2002) Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity. Proc. Natl. Acad. Sci. USA 99, 16412-16418. (Pubitemid 35470987)
212. Sakata E., Yamaguchi Y., Kurimoto E., et al. (2003) Parkin binds the Rpn10 subunit of 26S proteasomes through its ubiquitin-like domain. EMBO Rep. 4, 301-306. (Pubitemid 36511728)
213. Salminen A., Ojala J., Kaarniranta K., Hiltunen M., and, Soininen H., (2011) Hsp90 regulates Tau pathology through co-chaperone complexes in Alzheimer's disease. Prog. Neurobiol. 93, 99-110.
214. Santos R. X., Correia S. C., Wang X., Perry G., Smith M. A., Moreira P. I., and, Zhu X., (2010) A synergistic dysfunction of mitochondrial fission/fusion dynamics and mitophagy in Alzheimer's disease. J. Alzheimers Dis. 20, S401-S412.
215. Schlossmacher M. G., Frosch M. P., Gai W. P., et al. (2002) Parkin localizes to the Lewy bodies of Parkinson disease and dementia with Lewy bodies. Am. J. Pathol. 160, 1655-1667. (Pubitemid 34525646)
216. Schmitz A., Schneider A., Kummer M. P., and, Herzog V., (2004) Endoplasmic reticulum-localized amyloid beta-peptide is degraded in the cytosol by two distinct degradation pathways. Traffic 5, 89-101. (Pubitemid 38196021)
217. Schwarz L. A., Hall B. J., and, Patrick G. N., (2010) Activity-dependent ubiquitination of GluA1 mediates a distinct AMPA receptor endocytosis and sorting pathway. J. Neurosci. 30, 16718-16729.
218. Selkoe D. J., (1999) Translating cell biology into therapeutic advances in Alzheimer's disease. Nature 399, A23-A31.
219. Shimura H., Hattori N., Kubo S., et al. (2000) Familial Parkinson disease gene product, parkin, is a ubiquitin-protein ligase. Nat. Genet. 25, 302-305. (Pubitemid 30437317)
220. Shimura H., Schlossmacher M. G., Hattori N., Frosch M. P., Trockenbacher A., Schneider R., Mizuno Y., Kosik K. S., and, Selkoe D. J., (2001) Ubiquitination of a new form of alpha-synuclein by parkin from human brain: implications for Parkinson's disease. Science 293, 263-269. (Pubitemid 32694735)
221. Shimura H., Schwartz D., Gygi S. P., and, Kosik K. S., (2004) CHIP-Hsc70 complex ubiquitinates phosphorylated Tau and enhances cell survival. J. Biol. Chem. 279, 4869-4876. (Pubitemid 38199082)
222. Shin Y., Klucken J., Patterson C., Hyman B. T., and, McLean P. J., (2005) The co-chaperone carboxyl terminus of Hsp70-interacting protein (CHIP) mediates alpha-synuclein degradation decisions between proteasomal and lysosomal pathways. J. Biol. Chem. 280, 23727-23734. (Pubitemid 40884855)
223. Shinbo Y., Taira T., Niki T., Iguchi-Ariga S. M., and, Ariga H., (2005) DJ-1 restores p53 transcription activity inhibited by Topors/p53BP3. Int. J. Oncol. 26, 641-648.
224. Sone J., Niwa J., Kawai K., Ishigaki S., Yamada S., Adachi H., Katsuno M., Tanaka F., Doyu M., and, Sobue G., (2010) Dorfin ameliorates phenotypes in a transgenic mouse model of amyotrophic lateral sclerosis. J. Neurosci. Res. 88, 123-135.
225. Springer W., and, Kahle P. J., (2011) Regulation of PINK1-Parkin-mediated mitophagy. Autophagy 7, 266-278.
226. Sroka K., Voigt A., Deeg S., Reed J. C., Schulz J. B., Bähr M., and, Kermer P., (2009) BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution. J. Neurochem. 111, 801-807.
227. Staropoli J. F., McDermott C., Martinat C., Schulman B., Demireva E., and, Abeliovich A., (2003) Parkin is a component of an SCF-like ubiquitin ligase complex and protects postmitotic neurons from kainate excitotoxicity. Neuron 37, 735-749. (Pubitemid 36288547)
228. Subramaniam S., Sixt K. M., Barrow R., and, Snyder S. H., (2009) Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity. Science 324, 1327-1330.
229. Subramaniam S., Mealer R. G., Sixt K. M., Barrow R. K., Usiello A., and, Snyder S. H., (2010) Rhes, a physiologic regulator of sumoylation, enhances cross-sumoylation between the basic sumoylation enzymes E1 and Ubc9. J. Biol. Chem. 285, 20428-20432.
230. Takahashi R., and, Imai Y., (2003) Pael receptor, endoplasmic reticulum stress, and Parkinson's disease. J. Neurol. 250, III25-III29.
231. Takami M., Nagashima Y., Sano Y., Ishihara S., Morishima-Kawashima M., Funamoto S., and, Ihara Y., (2009) Gamma-Secretase: successive tripeptide and tetrapeptide release from the transmembrane domain of beta-carboxyl terminal fragment. J. Neurosci. 14, 13042-13052.
232. Takeuchi H., Niwa J., Hishikawa N., Ishigaki S., Tanaka F., Doyu M., and, Sobue G., (2004) Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis. J. Neurochem. 89, 64-72. (Pubitemid 38420675)
233. Tanji K., Kamitani T., Mori F., Kakita A., Takahashi H., and, Wakabayashi K., (2010) TRIM9, a novel brain-specific E3 ubiquitin ligase, is repressed in the brain of Parkinson's disease and dementia with Lewy bodies. Neurobiol. Dis. 38, 210-218.
234. Tetzlaff J. E., Putcha P., Outeiro T. F., Ivanov A., Berezovska O., Hyman B. T., and, McLean P. J., (2008) CHIP targets toxic alpha-Synuclein oligomers for degradation. J. Biol. Chem. 283, 17962-17968.
235. Todi S. V., and, Paulson H. L., (2007) Dangerous liaisons: polyglutamine meets HMGB. Nat. Cell Biol. 9, 359-361. (Pubitemid 46511066)
236. Todi S. V., Laco M. N., Winborn B. J., Travis S. M., Wen H. M., and, Paulson H. L., (2007) Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity. J. Biol. Chem. 282, 29348-29358. (Pubitemid 350043336)
237. Tsai Y. C., Fishman P. S., Thakor N. V., and, Oyler G. A., (2003) Parkin facilitates the elimination of expanded polyglutamine proteins and leads to preservation of proteasome function. J. Biol. Chem. 278, 22044-22055. (Pubitemid 36792614)
238. Tsang A. H., Lee Y. I., Ko H. S., Savitt J. M., Pletnikova O., Troncoso J. C., Dawson V. L., Dawson T. M., and, Chung K. K., (2009) S-Nitrosylation of XIAP compromises neuronal survival in Parkinson's disease. Proc. Natl. Acad. Sci. USA 106, 4900-4905.
239. Tsuang D. W., Riekse R. G., Purganan K. M., David A. C., Montine T. J., Schellenberg G. D., Steinbart E. J., Petrie E. C., Bird T. D., and, Leverenz J. B., (2006) Lewy body pathology in late-onset familial Alzheimer's disease: a clinicopathological case series. J. Alzheimers Dis. 9, 235-242. (Pubitemid 44253341)
240. Tsvetkov P., Adamovich Y., Elliott E., and, Shaul Y., (2011) E3 ligase STUB1/CHIP regulates NAD(P)H:quinone oxidoreductase 1 (NQO1) accumulation in aged brain, a process impaired in certain Alzheimer disease patients. J. Biol. Chem. 286, 8839-8845.
241. Uchikado H., Lin W. L., DeLucia M. W., and, Dickson D. W., (2006) Alzheimer disease with amygdale Lewy bodies: a distinct form of alpha-synucleinopathy. J. Neuropathol. Exp. Neurol. 65, 685-697. (Pubitemid 44297259)
242. Ulusoy A., and, Kirik D., (2008) Can overexpression of parkin provide a novel strategy for neuroprotection in Parkinson's disease? Exp. Neurol. 212, 258-260.
243. Um J. W., Min D. S., Rhim H., Kim J., Paik S. R., and, Chung K. C., (2006) Parkin ubiquitinates and promotes the degradation of RanBP2. J. Biol. Chem. 281, 3595-3603. (Pubitemid 43845977)
244. Um J. W., Im E., Lee H. J., et al. (2010) Parkin directly modulates 26S proteasome activity. J. Neurosci. 30, 11805-11814.
245. Umeda T., Tomiyama T., Sakama N., Tanaka S., Lambert M. P., Klein W. L., and, Mori H., (2011) Intraneuronal amyloid β oligomers cause cell death via endoplasmic reticulum stress, endosomal/lysosomal leakage, and mitochondrial dysfunction in vivo. J. Neurosci. Res. 89, 1031-1042.
246. Upadhya S. C., Ding L., Smith T. K., and, Hegde A. N., (2006) Differential regulation of proteasome activity in the nucleus and the synaptic terminals. Neurochem. Int. 48, 296-305. (Pubitemid 43177151)
247. Urushitani M., Kurisu J., Tateno M., Hatakeyama S., Nakayama K., Kato S., and, Takahashi R., (2004) CHIP promotes proteasomal degradation of familial ALS-linked mutant SOD1 by ubiquitinating Hsp/Hsc70. J. Neurochem. 90, 231-244. (Pubitemid 38879177)
248. Urushitani M., Sato T., Bamba H., Hisa Y., and, Tooyama I., (2010) Synergistic effect between proteasome and autophagosome in the clearance of polyubiquitinated TDP-43. J. Neurosci. Res. 88, 784-797.
249. Varshavsky A., Byrd C., Davydov I. V., et al. (1998) The N-end rule pathway, in Ubiquitin and the Biology of the Cell (, Peters J.-M., Harris J. R., and, Finley D., eds), pp. 223-278. Plenum Publishing Company Ltd, London.
250. Veeriah S., Morris L., Solit D., and, Chan T. A., (2010) The familial Parkinson disease gene PARK2 is a multisite tumor suppressor on chromosome 6q25.2-27 that regulates cyclin E. Cell Cycle 9, 1451-1452.
251. Vives-Bauza C., Zhou C., Huang Y., et al. (2010) PINK1-dependent recruitment of Parkin to mitochondria in mitophagy. Proc. Natl. Acad. Sci. USA 107, 378-383.
252. Vossel K. A., Zhang K., Brodbeck J., Daub A. C., Sharma P., Finkbeiner S., Cui B., and, Mucke L., (2010) Tau reduction prevents Abeta-induced defects in axonal transport. Science 330, 198.
253. Wang I. F., Wu L. S., and, Shen C. K., (2008a) TDP-43: an emerging new player in neurodegenerative diseases. Trends Mol. Med. 14, 479-485.
254. Wang J., Wang C. E., Orr A., Tydlacka S., Li S. H., and, Li X. J., (2008b) Impaired ubiquitin-proteasome system activity in the synapses of Huntington's disease mice. J. Cell Biol. 180, 1177-1189. (Pubitemid 351468473)
255. West A. B., Gonzalez-de-Chavez F., Wilkes K., O'Farrell C., and, Farrer M. J., (2003) Parkin is not regulated by the unfolded protein response in human neuroblastoma cells. Neurosci. Lett. 341, 139-142. (Pubitemid 36398488)
256. Whitworth A. J., Theodore D. A., Greene J. C., Benes H., Wes P. D., and, Pallanck L. J., (2005) Increased glutathione S-transferase activity rescues dopaminergic neuron loss in a Drosophila model of Parkinson's disease. Proc. Natl. Acad. Sci. USA 102, 8024-8029. (Pubitemid 40781056)
257. Wickner S., Maurizi M. R., and, Gottesman S., (1999) Posttranslational quality control: folding, refolding, and degrading proteins. Science 286, 1888-1893. (Pubitemid 129515889)
258. Williams A. J., Knutson T. M., Colomer Gould V. F., and, Paulson H. L., (2009) In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis. Neurobiol. Dis. 33, 342-353.
259. Winklhofer K. F., (2007) The parkin protein as a therapeutic target in Parkinson's disease. Expert. Opin. Ther. Targets 11, 1543-1552. (Pubitemid 350285004)
260. Winklhofer K. F., and, Haass C., (2010) Mitochondrial dysfunction in Parkinson's disease. Biochim. Biophys. Acta 1802, 29-44.
261. Xu Z., Kohli E., Devlin K. I., Bold M., Nix J. C., and, Misra S., (2008) Interactions between the quality control ubiquitin ligase CHIP and ubiquitin conjugating enzymes. BMC Struct. Biol. 8, 26.
262. Yamada M., Mizuno Y., and, Mochizuki H., (2005) Parkin gene therapy for alpha-synucleinopathy: a rat model of Parkinson's disease. Hum. Gene Ther. 16, 262-270. (Pubitemid 40344217)
263. Yan J., Wang J., Li Q., Hwang J. R., Patterson C., and, Zhang H., (2003) At CHIP, a U-box-containing E3 ubiquitin ligase plays a critical role in temperature stress tolerance in Arabidopsis. Plant Physiol. 132, 861-869. (Pubitemid 36715085)
264. Yang Y., Nishimura I., Imai Y., Takahashi R., and, Lu B., (2003) Parkin suppresses dopaminergic neuron-selective neurotoxicity induced by Pael-R in Drosophila. Neuron 37, 911-924. (Pubitemid 36398298)
265. Yang F., Jiang Q., Zhao J., Ren Y., Sutton M. D., and, Feng J., (2005) Parkin stabilizes microtubules through strong binding mediated by three independent domains. J. Biol. Chem. 280, 17154-17162. (Pubitemid 41389181)
266. Yang H., Zhong X., Ballar P., Luo S., Shen Y., Rubinsztein D. C., Monteiro M. J., and, Fang S., (2007) Ubiquitin ligase Hrd1 enhances the degradation and suppresses the toxicity of polyglutamine-expanded huntingtin. Exp. Cell Res. 313, 538-550. (Pubitemid 46188633)
267. Yang H., Liu C., Zhong Y., Luo S., Monteiro M. J., and, Fang S., (2010) Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP. PLoS ONE 5, e8905.
268. Yao D., Gu Z., Nakamura T., Shi Z. Q., Ma Y., Gaston B., Palmer L. A., and, Rockenstein E. M., (2004) Nitrosative stress linked to sporadic Parkinson's disease: S-nitrosylation of parkin regulates its E3 ubiquitin ligase activity. Proc. Natl. Acad. Sci. USA 101, 10810-10814. (Pubitemid 38955824)
269. Ying Z., Wang H., Fan H., Zhu X., Zhou J., Fei E., and, Wang G., (2009) Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation. Hum. Mol. Genet. 18, 4268-4281.
270. Young J. C., Moarefi I., and, Hartl F. U., (2001) Hsp90: a specialized but essential protein-folding tool. J. Cell Biol. 154, 267-273. (Pubitemid 34286138)
271. Younger J. M., Ren H. Y., Chen L., Fan C. Y., Fields A., Patterson C., and, Cyr D. M., (2004) A foldable CFTR{Delta}F508 biogenic intermediate accumulates upon inhibition of the Hsc70-CHIP E3 ubiquitin ligase. J. Cell Biol. 167, 1075-1085. (Pubitemid 40066623)
272. Yu F., and, Zhou J., (2008) Parkin is ubiquitinated by Nrdp1 and abrogates Nrdp1-induced oxidative stress. Neurosci. Lett. 440, 4-8.
273. Zhang Y., Gao J., Chung K. K., Huang H., Dawson V. L., and, Dawson T. M., (2000) Parkin functions as an E2-dependent ubiquitin- protein ligase and promotes the degradation of the synaptic vesicle-associated protein, CDCrel-1. Proc. Natl. Acad. Sci. USA 97, 13354-13359.
274. Zhang G. R., Cheng X. R., Zhou W. X., and, Zhang Y. X., (2008a) Age-related expression of STUB1 in senescence-accelerated mice and its response to anti-Alzheimer's disease traditional Chinese medicine. Neurosci. Lett. 438, 371-375.
275. Zhang Y. J., Xu Y. F., Liu X. H., Li D., Yin J., Liu Y. H., Chen X. Q., and, Wang J. Z., (2008b) Carboxyl terminus of heat-shock cognate 70-interacting protein degrades Tau regardless its phosphorylation status without affecting the spatial memory of the rats. J. Neural Transm. 115, 483-491.
276. Zhang L., Haraguchi S., Koda T., Hashimoto K., and, Nakagawara A., (2011) Muscle atrophy and motor neuron degeneration in human NEDL1 transgenic mice. J. Biomed. Biotechnol. 2011, 831092.
277. Zheng N., Schulman B. A., Song L., et al. (2002) Structure of the Cul1-Rbx1-Skp1-F box Skp2 SCF ubiquitin ligase complex. Nature 416, 703-709. (Pubitemid 34429140)
278. Zuccato C., Valenza M., and, Cattaneo E., (2010) Molecular mechanisms and potential therapeutical targets in Huntington's disease. Physiol. Rev. 90, 905-981.
279. Zucchelli S., Marcuzzi F., Codrich M., et al. (2011) Tumor Necrosis Factor Receptor-associated Factor 6 (TRAF6) Associates with Huntingtin Protein and Promotes Its Atypical Ubiquitination to Enhance Aggregate Formation. J. Biol. Chem. 286, 25108-25117.