Quality Control Compartments Coming of Age

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Volumn 13, Issue 5, 2012, Pages 635-642

  Ben Gedalya, Tziona ^a   Cohen, Ehud ^a  

^a HEBREW UNIVERSITY OF JERUSALEM   (Israel)

Author keywords

Aging; Degradation; Deposition site; Neurodegeneration; Protein aggregation; Quality control

Indexed keywords

ALPHA SYNUCLEIN; AMYLOID BETA PROTEIN; CHAPERONE; CYCLOSPORIN A; HUNTINGTIN; NOCODAZOLE; PROTEASOME; SOMATOMEDIN C RECEPTOR; TAU PROTEIN; TRANSMEMBRANE CONDUCTANCE REGULATOR;

AGING; ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; AUTOPHAGY; CELL COMPARTMENTALIZATION; HUMAN; HUNTINGTON CHOREA; LEWY BODY; NEUROTOXICITY; NONHUMAN; PARKINSON DISEASE; PRION DISEASE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN DEGRADATION; PROTEIN SYNTHESIS; QUALITY CONTROL; REVIEW;

AGING; ANIMALS; BIOLOGICAL TRANSPORT; BRAIN; CAENORHABDITIS ELEGANS; DROSOPHILA MELANOGASTER; HUMANS; INSULIN-LIKE GROWTH FACTOR I; MODELS, BIOLOGICAL; NEURODEGENERATIVE DISEASES; NEURONS; PEPTIDES; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEINS; RATS;

EID: 84859622560     PISSN: 13989219     EISSN: 16000854     Source Type: Journal    
DOI: 10.1111/j.1600-0854.2012.01330.x     Document Type: Review

References (74)

1. Balch WE, Morimoto RI, Dillin A, Kelly JW. Adapting proteostasis for disease intervention. Science 2008;319:916-919.
2. Hartl FU, Hayer-Hartl M. Converging concepts of protein folding in vitro and in vivo. Nat Struct Mol Biol 2009;16:574-581.
3. Matus S, Glimcher LH, Hetz C. Protein folding stress in neurodegenerative diseases: a glimpse into the ER. Curr Opin Cell Biol 2011;23:239-252.
4. Ciechanover A. The ubiquitin proteolytic system: from a vague idea, through basic mechanisms, and onto human diseases and drug targeting. Neurology 2006;66(2 Suppl. 1):S7-S19.
5. Tyedmers J, Mogk A, Bukau B. Cellular strategies for controlling protein aggregation. Nat Rev 2010;11:777-788.
6. Arias E, Cuervo AM. Chaperone-mediated autophagy in protein quality control. Curr Opin Cell Biol 2011;23:184-189.
7. Kopito RR, Ron D. Conformational disease. Nat Cell Biol 2000;2:E207-E209.
8. Carrell RW, Lomas DA. Conformational disease. Lancet 1997;350:134-138.
9. Selkoe DJ. Folding proteins in fatal ways. Nature 2003;426:900-904.
10. Bates G. Huntingtin aggregation and toxicity in Huntington's disease. Lancet 2003;361:1642-1644.
11. Ticozzi N, Tiloca C, Morelli C, Colombrita C, Poletti B, Doretti A, Maderna L, Messina S, Ratti A, Silani V. Genetics of familial amyotrophic lateral sclerosis. Arch Ital Biol 2011;149:65-82.
12. Aguzzi A, Calella AM. Prions: protein aggregation and infectious diseases. Physiol Rev 2009;89:1105-1152.
13. Ciechanover A, Brundin P. The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg. Neuron 2003;40:427-446.
14. Bieschke J, Cohen E, Murray A, Dillin A, Kelly JW. A kinetic assessment of the C. elegans amyloid disaggregation activity enables uncoupling of disassembly and proteolysis. Protein Sci 2009;18:2231-2241.
15. Leissring MA, Farris W, Chang AY, Walsh DM, Wu X, Sun X, Frosch MP, Selkoe DJ. Enhanced proteolysis of beta-amyloid in APP transgenic mice prevents plaque formation, secondary pathology, and premature death. Neuron 2003;40:1087-1093.
16. Wong ES, Tan JM, Soong WE, Hussein K, Nukina N, Dawson VL, Dawson TM, Cuervo AM, Lim KL. Autophagy-mediated clearance of aggresomes is not a universal phenomenon. Hum Mol Genet 2008;17:2570-2582.
17. Webb JL, Ravikumar B, Atkins J, Skepper JN, Rubinsztein DC. Alpha-synuclein is degraded by both autophagy and the proteasome. J Biol Chem 2003;278:25009-25013.
18. Soto C. Unfolding the role of protein misfolding in neurodegenerative diseases. Nat Rev 2003;4:49-60.
19. Selkoe DJ. Alzheimer's disease. Cold Spring Harb Perspect Biol 2011;3:pii: a004457. doi: 10.1101/cshperspect.a004457.
20. Jankowsky JL, Fadale DJ, Anderson J, Xu GM, Gonzales V, Jenkins NA, Copeland NG, Lee MK, Younkin LH, Wagner SL, Younkin SG, Borchelt DR. Mutant presenilins specifically elevate the levels of the 42 residue beta-amyloid peptide in vivo: evidence for augmentation of a 42-specific gamma secretase. Hum Mol Genet 2004;13:159-170.
21. Lee VM, Trojanowski JQ. Mechanisms of Parkinson's disease linked to pathological alpha-synuclein: new targets for drug discovery. Neuron 2006;52:33-38.
22. Marsden CD. Parkinson's disease. Lancet 1990;335:948-952.
23. Holdorff B. Friedrich Heinrich Lewy (1885-1950) and his work. J Hist Neurosci 2002;11:19-28.
24. Spillantini MG, Schmidt ML, Lee VM, Trojanowski JQ, Jakes R, Goedert M. Alpha-synuclein in Lewy bodies. Nature 1997;388:839-840.
25. McNaught KS, Shashidharan P, Perl DP, Jenner P, Olanow CW. Aggresome-related biogenesis of Lewy bodies. Eur J Neurosci 2002;16:2136-2148.
26. Love S, Saitoh T, Quijada S, Cole GM, Terry RD. Alz-50, ubiquitin and tau immunoreactivity of neurofibrillary tangles, Pick bodies and Lewy bodies. J Neuropathol Exp Neurol 1988;47:393-405.
27. Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J, Vonsattel JP, Aronin N, DiFiglia M. Huntingtin localization in brains of normal and Huntington's disease patients. Ann Neurol 1997;42:604-612.
28. Prusiner SB. Prions. Proc Natl Acad Sci U S A 1998;95:13363-13383.
29. Lee SJ, Desplats P, Sigurdson C, Tsigelny I, Masliah E. Cell-to-cell transmission of non-prion protein aggregates. Nat Rev Neurol 2010;6:702-706.
30. Meyer-Luehmann M, Coomaraswamy J, Bolmont T, Kaeser S, Schaefer C, Kilger E, Neuenschwander A, Abramowski D, Frey P, Jaton AL, Vigouret JM, Paganetti P, Walsh DM, Mathews PM, Ghiso J et al. Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science 2006;313:1781-1784.
31. Westermark GT, Westermark P. Prion-like aggregates: infectious agents in human disease. Trends Mol Med 2010;16:501-507.
32. Cipriani G, Dolciotti C, Picchi L, Bonuccelli U. Alzheimer and his disease: a brief history. Neurol Sci 2011;32:275-279.
33. Alzheimer A. Über eine eigenartige Erkrankung der Hirnrinde. Allgemeine Zeitschrift für Psychiatrie 1907;64:146-148.
34. Wojcik C, Schroeter D, Wilk S, Lamprecht J, Paweletz N. Ubiquitin-mediated proteolysis centers in HeLa cells: indication from studies of an inhibitor of the chymotrypsin-like activity of the proteasome. Eur J Cell Biol 1996;71:311-318.
35. Johnston JA, Ward CL, Kopito RR. Aggresomes: a cellular response to misfolded proteins. J Cell Biol 1998;143:1883-1898.
36. Kristiansen M, Messenger MJ, Klohn PC, Brandner S, Wadsworth JD, Collinge J, Tabrizi SJ. Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem 2005;280:38851-38861.
37. Mishra RS, Bose S, Gu Y, Li R, Singh N. Aggresome formation by mutant prion proteins: the unfolding role of proteasomes in familial prion disorders. J Alzheimers Dis 2003;5:15-23.
38. Waelter S, Boeddrich A, Lurz R, Scherzinger E, Lueder G, Lehrach H, Wanker EE. Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation. Mol Biol Cell 2001;12:1393-1407.
39. Tanaka M, Kim YM, Lee G, Junn E, Iwatsubo T, Mouradian MM. Aggresomes formed by alpha-synuclein and synphilin-1 are cytoprotective. J Biol Chem 2004;279:4625-4631.
40. Garcia-Mata R, Bebok Z, Sorscher EJ, Sztul ES. Characterization and dynamics of aggresome formation by a cytosolic GFP-chimera. J Cell Biol 1999;146:1239-1254.
41. Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB. Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature 1989;338:342-345.
42. Yamazaki M, Oyanagi K, Mori O, Kitamura S, Ohyama M, Terashi A, Kitamoto T, Katayama Y. Variant Gerstmann-Straussler syndrome with the P105L prion gene mutation: an unusual case with nigral degeneration and widespread neurofibrillary tangles. Acta Neuropathol (Berl) 1999;98:506-511.
43. Barik S. Immunophilins: for the love of proteins. Cell Mol Life Sci 2006;63:2889-2900.
44. Cohen E, Taraboulos A. Scrapie-like prion protein accumulates in aggresomes of cyclosporin A-treated cells. EMBO J 2003;22:404-417.
45. Ben-Gedalya T, Lyakhovetsky R, Yedidia Y, Bejerano-Sagie M, Kogan NM, Karpuj MV, Kaganovich D, Cohen E. Cyclosporin-A-induced prion protein aggresomes are dynamic quality-control cellular compartments. J Cell Sci 2011;124:1891-1902.
46. Taylor JP, Tanaka F, Robitschek J, Sandoval CM, Taye A, Markovic-Plese S, Fischbeck KH. Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein. Hum Mol Genet 2003;12:749-757.
47. Kaganovich D, Kopito R, Frydman J. Misfolded proteins partition between two distinct quality control compartments. Nature 2008;454:1088-1095.
48. Wong E, Cuervo AM. Autophagy gone awry in neurodegenerative diseases. Nat Neurosci 2010;13:805-811.
49. Katzman R, Brown T, Fuld P, Thal L, Davies P, Terry R. Significance of neurotransmitter abnormalities in Alzheimer's disease. Res Publ Assoc Res Nerv Ment Dis 1986;64:279-286.
50. Terry RD, Masliah E, Salmon DP, Butters N, DeTeresa R, Hill R, Hansen LA, Katzman R. Physical basis of cognitive alterations in Alzheimer's disease: synapse loss is the major correlate of cognitive impairment. Ann Neurol 1991;30:572-580.
51. Haass C, Selkoe DJ. Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide. Nat Rev 2007;8:101-112.
52. Shankar GM, Li S, Mehta TH, Garcia-Munoz A, Shepardson NE, Smith I, Brett FM, Farrell MA, Rowan MJ, Lemere CA, Regan CM, Walsh DM, Sabatini BL, Selkoe DJ. Amyloid-beta protein dimers isolated directly from Alzheimer's brains impair synaptic plasticity and memory. Nat Med 2008;14:837-842.
53. O'Nuallain B, Freir DB, Nicoll AJ, Risse E, Ferguson N, Herron CE, Collinge J, Walsh DM. Amyloid beta-protein dimers rapidly form stable synaptotoxic protofibrils. J Neurosci 2010;30:14411-14419.
54. Meyer-Luehmann M, Spires-Jones TL, Prada C, Garcia-Alloza M, de Calignon A, Rozkalne A, Koenigsknecht-Talboo J, Holtzman DM, Bacskai BJ, Hyman BT. Rapid appearance and local toxicity of amyloid-beta plaques in a mouse model of Alzheimer's disease. Nature 2008;451:720-724.
55. Shorter J, Lindquist S. Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformers. Science 2004;304:1793-1797.
56. Behrends C, Langer CA, Boteva R, Bottcher UM, Stemp MJ, Schaffar G, Rao BV, Giese A, Kretzschmar H, Siegers K, Hartl FU. Chaperonin TRiC promotes the assembly of polyQ expansion proteins into nontoxic oligomers. Mol Cell 2006;23:887-897.
57. Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004;431:805-810.
58. Assayag K, Yakunin E, Loeb V, Selkoe DJ, Sharon R. Polyunsaturated fatty acids induce alpha-synuclein-related pathogenic changes in neuronal cells. Am J Pathol 2007;171:2000-2011.
59. Amaducci L, Tesco G. Aging as a major risk for degenerative diseases of the central nervous system. Curr Opin Neurol 1994;7:283-286.
60. Cohen E, Bieschke J, Perciavalle RM, Kelly JW, Dillin A. Opposing activities protect against age-onset proteotoxicity. Science 2006;313:1604-1610.
61. Hsu AL, Murphy CT, Kenyon C. Regulation of aging and age-related disease by DAF-16 and heat-shock factor. Science 2003;300:1142-1145.
62. Morley JF, Brignull HR, Weyers JJ, Morimoto RI. The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans. Proc Natl Acad Sci U S A 2002;99:10417-10422.
63. Teixeira-Castro A, Ailion M, Jalles A, Brignull HR, Vilaca JL, Dias N, Rodrigues P, Oliveira JF, Neves-Carvalho A, Morimoto RI, Maciel P. Neuron-specific proteotoxicity of mutant ataxin-3 in C. elegans: rescue by the DAF-16 and HSF-1 pathways. Hum Mol Genet 2011;20:2996-3009.
64. Zhang T, Mullane PC, Periz G, Wang J. TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling. Hum Mol Genet 2011;20:1952-1965.
65. Cohen E, Paulsson JF, Blinder P, Burstyn-Cohen T, Du D, Estepa G, Adame A, Pham HM, Holzenberger M, Kelly JW, Masliah E, Dillin A. Reduced IGF-1 signaling delays age-associated proteotoxicity in mice. Cell 2009;139:1157-1169.
66. Freude S, Hettich MM, Schumann C, Stohr O, Koch L, Kohler C, Udelhoven M, Leeser U, Muller M, Kubota N, Kadowaki T, Krone W, Schroder H, Bruning JC, Schubert M. Neuronal IGF-1 resistance reduces Abeta accumulation and protects against premature death in a model of Alzheimer's disease. FASEB J 2009;23:3315-3324.
67. Kenyon C, Chang J, Gensch E, Rudner A, Tabtiang R. A C. elegans mutant that lives twice as long as wild type. Nature 1993;366:461-464.
68. Campion D, Brice A, Dumanchin C, Puel M, Baulac M, De La Sayette V, Hannequin D, Duyckaerts C, Michon A, Martin C, Moreau V, Penet C, Martinez M, Clerget-Darpoux F, Agid Y et al. A novel presenilin 1 mutation resulting in familial Alzheimer's disease with an onset age of 29 years. Neuroreport 1996;7:1582-1584.
69. Jankowsky JL, Slunt HH, Ratovitski T, Jenkins NA, Copeland NG, Borchelt DR. Co-expression of multiple transgenes in mouse CNS: a comparison of strategies. Biomol Eng 2001;17:157-165.
70. Holcomb L, Gordon MN, McGowan E, Yu X, Benkovic S, Jantzen P, Wright K, Saad I, Mueller R, Morgan D, Sanders S, Zehr C, O'Campo K, Hardy J, Prada CM et al. Accelerated Alzheimer-type phenotype in transgenic mice carrying both mutant amyloid precursor protein and presenilin 1 transgenes. Nat Med 1998;4:97-100.
71. Cohen E, Du D, Joyce D, Kapernick EA, Volovik Y, Kelly JW, Dillin A. Temporal requirements of insulin/IGF-1 signaling for proteotoxicity protection. Aging Cell 2010;9:126-134.
72. LaFerla FM, Green KN, Oddo S. Intracellular amyloid-beta in Alzheimer's disease. Nat Rev 2007;8:499-509.
73. Kordower JH, Chu Y, Hauser RA, Freeman TB, Olanow CW. Lewy body-like pathology in long-term embryonic nigral transplants in Parkinson's disease. Nat Med 2008;14:504-506.
74. Li JY, Englund E, Holton JL, Soulet D, Hagell P, Lees AJ, Lashley T, Quinn NP, Rehncrona S, Bjorklund A, Widner H, Revesz T, Lindvall O, Brundin P. Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation. Nat Med 2008;14:501-503.