CoChat
Sign Up
Cited by 251

The DOI matches its Crossref record; 14 of the 14 references it cites were found in a registry or an index.

Are synucleinopathies prion-like disorders?

Angot, Elodie, Steiner, Jennifer A, Hansen, Christian, Li, Jia-Yi, Brundin, Patrik·2010·The Lancet Neurology
DOI 10.1016/s1474-4422(10)70213-1
Open this paper in CoChat — free account

Abstract

A shared neuropathological feature of idiopathic Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy is the development of intracellular aggregates of α-synuclein that gradually engage increasing parts of the nervous system. The pathogenetic mechanisms underlying these neurodegenerative disorders, however, are unknown. Several studies have highlighted similarities between classic prion diseases and these neurological proteinopathies. Specifically, identification of Lewy bodies in fetal mesencephalic neurons transplanted in patients with Parkinson's disease raised the hypothesis that α-synuclein, the main component of Lewy bodies, could be transmitted from the host brain to a graft of healthy neurons. These results and others have led to the hypothesis that a prion-like mechanism might underlie progression of synucleinopathy within the nervous system. We review experimental findings showing that misfolded α-synuclein can transfer between cells and, once transferred into a new cell, can act as a seed that recruits endogenous α-synuclein, leading to formation of larger aggregates. This model suggests that strategies aimed at prevention of cell-to-cell transfer of α-synuclein could retard progression of symptoms in Parkinson's disease and other synucleinopathies.

Overview

Integrity signals

Registry

Crossref · 10.1016/s1474-4422(10)70213-1

Cites

14 of 14 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Verification

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • Oligomeric α-Syn species appear prone to uptake and spread from cell-to-cell in a prion-like manner, leading to templated misfolding of native α-Syn and formation of aggregates throughout the brain.

    “Particularly, the oligomeric α-Syn species appear to be prone to uptake and spread from cell-to-cell in a prion-like manner, leading to the templated misfolding of the native forms of α-Syn and formation of α-Syn aggregates throughout the brain, which are the defining pathological features of Parkinson’s disease (PD) and dementia with Lewy body (DLB) [12–19].”

    From LRP1 is a neuronal receptor for α-synuclein uptake and spread (2022) · cites this paper as [13]

Citations

Cites

Flagged first, then by citations

Research works printed but not linked: 104

  1. Uversky, 2008, α-synuclein misfolding and neurodegenerative diseases, Curr Protein Pept Sci, 9, 507, 10.2174/138920308785915218 · DOI
  2. Spillantini, 1997, α-synuclein in Lewy bodies, Nature, 388, 839, 10.1038/42166 · DOI · Paper page
  3. Aguzzi, 2009, Cell biology: beyond the prion principle, Nature, 459, 924, 10.1038/459924a · DOI
  4. Aguzzi, 2009, The transcellular spread of cytosolic amyloids, prions, and prionoids, Neuron, 64, 783, 10.1016/j.neuron.2009.12.016 · DOI · Paper page
  5. Angot, 2009, Dissecting the potential molecular mechanisms underlying α-synuclein cell-to-cell transfer in Parkinson's disease, Parkinsonism Relat Disord, 15, S143, 10.1016/S1353-8020(09)70802-8 · DOI
  6. Frost, 2010, Prion-like mechanisms in neurodegenerative diseases, Nat Rev Neurosci, 11, 155, 10.1038/nrn2786 · DOI · Paper page
  7. Olanow, 2009, Is Parkinson's disease a prion disorder?, Proc Natl Acad Sci USA, 106, 12571, 10.1073/pnas.0906759106 · DOI
  8. Goedert, 2010, The propagation of prion-like protein inclusions in neurodegenerative diseases, Trends Neurosci, 33, 317, 10.1016/j.tins.2010.04.003 · DOI
  9. Prusiner, 1982, Novel proteinaceous infectious particles cause scrapie, Science, 216, 136, 10.1126/science.6801762 · DOI
  10. Prusiner, 2001, Shattuck lecture: neurodegenerative diseases and prions, N Engl J Med, 344, 1516, 10.1056/NEJM200105173442006 · DOI
  11. Appleby, 2009, Characteristics of established and proposed sporadic Creutzfeldt-Jakob disease variants, Arch Neurol, 66, 208, 10.1001/archneurol.2008.533 · DOI
  12. Diener, 1982, Viroids and prions, Proc Natl Acad Sci USA, 79, 5220, 10.1073/pnas.79.17.5220 · DOI
  13. Hsiao, 1989, Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome, Nature, 338, 342, 10.1038/338342a0 · DOI
  14. Medori, 1992, Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene, N Engl J Med, 326, 444, 10.1056/NEJM199202133260704 · DOI
  15. Jackson, 2009, Spontaneous generation of prion infectivity in fatal familial insomnia knockin mice, Neuron, 63, 438, 10.1016/j.neuron.2009.07.026 · DOI
  16. Steele, 2009, Context dependent neuroprotective properties of prion protein (PrP), Prion, 3, 240, 10.4161/pri.3.4.10135 · DOI
  17. Si, 2010, Aplysia CPEB can form prion-like multimers in sensory neurons that contribute to long-term facilitation, Cell, 140, 421, 10.1016/j.cell.2010.01.008 · DOI
  18. Prusiner, 1993, Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies, Proc Natl Acad Sci USA, 90, 10608, 10.1073/pnas.90.22.10608 · DOI
  19. Sailer, 1994, No propagation of prions in mice devoid of PrP, Cell, 77, 967, 10.1016/0092-8674(94)90436-7 · DOI
  20. Weissmann, 1994, PrP-deficient mice are resistant to scrapie, Ann N Y Acad Sci, 724, 235, 10.1111/j.1749-6632.1994.tb38913.x · DOI
  21. Riek, 1996, NMR structure of the mouse prion protein domain PrP(121-321), Nature, 382, 180, 10.1038/382180a0 · DOI
  22. Wille, 2009, Natural and synthetic prion structure from X-ray fiber diffraction, Proc Natl Acad Sci USA, 106, 16990, 10.1073/pnas.0909006106 · DOI
  23. Tanaka, 2006, The physical basis of how prion conformations determine strain phenotypes, Nature, 442, 585, 10.1038/nature04922 · DOI
  24. Yam, 2010, The octarepeat region of the prion protein is conformationally altered in PrP(Sc), PLoS One, 5, e9316, 10.1371/journal.pone.0009316 · DOI
  25. Bergasa-Caceres, 2010, Low entropic barrier to the hydrophobic collapse of the prion protein: effects of intermediate states and conformational flexibility, J Phys Chem A, 114, 6978, 10.1021/jp909944p · DOI
  26. Colby, 2009, Design and construction of diverse mammalian prion strains, Proc Natl Acad Sci USA, 106, 20417, 10.1073/pnas.0910350106 · DOI
  27. Alais, 2008, Mouse neuroblastoma cells release prion infectivity associated with exosomal vesicles, Biol Cell, 100, 603, 10.1042/BC20080025 · DOI
  28. Février, 2004, Cells release prions in association with exosomes, Proc Natl Acad Sci USA, 101, 9683, 10.1073/pnas.0308413101 · DOI
  29. Gousset, 2009, Prions hijack tunnelling nanotubes for intercellular spread, Nat Cell Biol, 11, 328, 10.1038/ncb1841 · DOI
  30. Jen, 2010, Neuronal low-density lipoprotein receptor-related protein 1 binds and endocytoses prion fibrils via receptor cluster 4, J Cell Sci, 123, 246, 10.1242/jcs.058099 · DOI
  31. Veith, 2009, Immunolocalisation of PrPSc in scrapie-infected N2a mouse neuroblastoma cells by light and electron microscopy, Eur J Cell Biol, 88, 45, 10.1016/j.ejcb.2008.08.001 · DOI
  32. Vella, 2007, Packaging of prions into exosomes is associated with a novel pathway of PrP processing, J Pathol, 211, 582, 10.1002/path.2145 · DOI
  33. Simons, 2009, Exosomes: vesicular carriers for intercellular communication, Curr Opin Cell Biol, 21, 575, 10.1016/j.ceb.2009.03.007 · DOI
  34. Février, 2005, Exosomes: a bubble ride for prions?, Traffic, 6, 10, 10.1111/j.1600-0854.2004.00247.x · DOI
  35. Davis, 2008, Membrane nanotubes: dynamic long-distance connections between animal cells, Nat Rev Mol Cell Biol, 9, 431, 10.1038/nrm2399 · DOI
  36. Mabbott, 2006, Prions and their lethal journey to the brain, Nat Rev Microbiol, 4, 201, 10.1038/nrmicro1346 · DOI
  37. Cohen, 1987, The pathogenesis and biochemistry of amyloidosis, J Pathol, 151, 1, 10.1002/path.1711510102 · DOI
  38. Halliday, 2010, The progression of pathology in Parkinson's disease, Ann N Y Acad Sci, 1184, 188, 10.1111/j.1749-6632.2009.05118.x · DOI
  39. Barone, 2009, The PRIAMO study: a multicenter assessment of nonmotor symptoms and their impact on quality of life in Parkinson's disease, Mov Disord, 24, 1641, 10.1002/mds.22643 · DOI · Paper page
  40. Olanow, 2004, Lewy-body formation is an aggresome-related process: a hypothesis, Lancet Neurol, 3, 496, 10.1016/S1474-4422(04)00827-0 · DOI
  41. Braak, 2002, Staging of the intracerebral inclusion body pathology associated with idiopathic Parkinson's disease (preclinical and clinical stages), J Neurol, 249, III/1, 10.1007/s00415-002-1301-4 · DOI
  42. Braak, 2003, Staging of brain pathology related to sporadic Parkinson's disease, Neurobiol Aging, 24, 197, 10.1016/S0197-4580(02)00065-9 · DOI · Paper page
  43. Braak, 2004, Stages in the development of Parkinson's disease-related pathology, Cell Tissue Res, 318, 121, 10.1007/s00441-004-0956-9 · DOI · Paper page
  44. Stefanova, 2009, Multiple system atrophy: an update, Lancet Neurol, 8, 1172, 10.1016/S1474-4422(09)70288-1 · DOI
  45. Uéda, 1993, Molecular cloning of cDNA encoding an unrecognized component of amyloid in Alzheimer disease, Proc Natl Acad Sci USA, 90, 11282, 10.1073/pnas.90.23.11282 · DOI
  46. Jakes, 1994, Identification of two distinct synucleins from human brain, FEBS Lett, 345, 27, 10.1016/0014-5793(94)00395-5 · DOI
  47. Krüger, 1998, Ala30Pro mutation in the gene encoding α-synuclein in Parkinson's disease, Nat Genet, 18, 106, 10.1038/ng0298-106 · DOI · Paper page
  48. Singleton, 2003, α-synuclein locus triplication causes Parkinson's disease, Science, 302, 841, 10.1126/science.1090278 · DOI · Paper page
  49. Chartier-Harlin, 2004, α-synuclein locus duplication as a cause of familial Parkinson's disease, Lancet, 364, 1167, 10.1016/S0140-6736(04)17103-1 · DOI · Paper page
  50. Zarranz, 2004, The new mutation, E46K, of α-synuclein causes Parkinson and Lewy body dementia, Ann Neurol, 55, 164, 10.1002/ana.10795 · DOI · Paper page
  51. El-Agnaf, 2003, α-synuclein implicated in Parkinson's disease is present in extracellular biological fluids, including human plasma, FASEB J, 17, 1945, 10.1096/fj.03-0098fje · DOI · Paper page
  52. El-Agnaf, 2006, Detection of oligomeric forms of α-synuclein protein in human plasma as a potential biomarker for Parkinson's disease, FASEB J, 20, 419, 10.1096/fj.03-1449com · DOI · Paper page
  53. Noguchi-Shinohara, 2009, CSF α-synuclein levels in dementia with Lewy bodies and Alzheimer's disease, Brain Res, 1251, 1, 10.1016/j.brainres.2008.11.055 · DOI
  54. Chu, 2007, Age-associated increases of α-synuclein in monkeys and humans are associated with nigrostriatal dopamine depletion: is this the target for Parkinson's disease?, Neurobiol Dis, 25, 134, 10.1016/j.nbd.2006.08.021 · DOI
  55. Hawkes, 2009, Parkinson's disease: the dual hit theory revisited, Ann N Y Acad Sci, 1170, 615, 10.1111/j.1749-6632.2009.04365.x · DOI · Paper page
  56. Daniel, 1992, Preliminary diagnosis of Parkinson's disease by olfactory bulb pathology, Lancet, 340, 186, 10.1016/0140-6736(92)93275-R · DOI
  57. Ubeda-Bañon, 2010, alpha-Synucleinopathy in the human olfactory system in Parkinson's disease: involvement of calcium-binding protein- and substance P-positive cells, Acta Neuropathol, 119, 723, 10.1007/s00401-010-0687-9 · DOI
  58. Duda, 1999, The expression of α-, β-, and γ-synucleins in olfactory mucosa from patients with and without neurodegenerative diseases, Exp Neurol, 160, 515, 10.1006/exnr.1999.7228 · DOI
  59. Braak, 2006, Gastric α-synuclein immunoreactive inclusions in Meissner's and Auerbach's plexuses in cases staged for Parkinson's disease-related brain pathology, Neurosci Lett, 396, 67, 10.1016/j.neulet.2005.11.012 · DOI · Paper page
  60. Chaudhuri, 2008, Early Parkinson's disease and non-motor issues, J Neurol, 255, 33, 10.1007/s00415-008-5006-1 · DOI
  61. Park, 2009, Non-motor symptoms in Parkinson's disease, J Neurol, 256, 293, 10.1007/s00415-009-5240-1 · DOI
  62. Maetzler, 2009, Progression of Parkinson's disease in the clinical phase: potential markers, Lancet Neurol, 8, 1158, 10.1016/S1474-4422(09)70291-1 · DOI
  63. Mendez, 2008, Dopamine neurons implanted into people with Parkinson's disease survive without pathology for 14 years, Nat Med, 14, 507, 10.1038/nm1752 · DOI
  64. Björklund, 2003, Neural transplantation for the treatment of Parkinson's disease, Lancet Neurol, 2, 437, 10.1016/S1474-4422(03)00442-3 · DOI
  65. Olanow, 2009, Dopaminergic transplantation for Parkinson's disease: current status and future prospects, Ann Neurol, 66, 591, 10.1002/ana.21778 · DOI
  66. Kordower, 2008, Transplanted dopaminergic neurons develop PD pathologic changes: a second case report, Mov Disord, 23, 2303, 10.1002/mds.22369 · DOI
  67. Chu, 2010, Lewy body pathology in fetal grafts, Ann N Y Acad Sci, 1184, 55, 10.1111/j.1749-6632.2009.05229.x · DOI
  68. Li, 2010, Characterization of Lewy body pathology in 12- and 16-year-old intrastriatal mesencephalic grafts surviving in a patient with Parkinson's disease, Mov Disord, 25, 1091, 10.1002/mds.23012 · DOI
  69. Brundin, 2008, Research in motion: the enigma of Parkinson's disease pathology spread, Nat Rev Neurosci, 9, 741, 10.1038/nrn2477 · DOI
  70. Elbaz, 2009, Professional exposure to pesticides and Parkinson disease, Ann Neurol, 66, 494, 10.1002/ana.21717 · DOI
  71. Gorell, 1998, The risk of Parkinson's disease with exposure to pesticides, farming, well water, and rural living, Neurology, 50, 1346, 10.1212/WNL.50.5.1346 · DOI
  72. Hatcher, 2008, Parkinson's disease and pesticides: a toxicological perspective, Trends Pharmacol Sci, 29, 322, 10.1016/j.tips.2008.03.007 · DOI
  73. Lee, 2002, Formation and removal of α-synuclein aggregates in cells exposed to mitochondrial inhibitors, J Biol Chem, 277, 5411, 10.1074/jbc.M105326200 · DOI
  74. Betarbet, 2000, Chronic systemic pesticide exposure reproduces features of Parkinson's disease, Nat Neurosci, 3, 1301, 10.1038/81834 · DOI
  75. Cannon, 2009, A highly reproducible rotenone model of Parkinson's disease, Neurobiol Dis, 34, 279, 10.1016/j.nbd.2009.01.016 · DOI
  76. Desplats, 2009, Inclusion formation and neuronal cell death through neuron-to-neuron transmission of α-synuclein, Proc Natl Acad Sci USA, 106, 13010, 10.1073/pnas.0903691106 · DOI · Paper page
  77. Miller, 2005, Absence of α-synuclein mRNA expression in normal and multiple system atrophy oligodendroglia, J Neural Transm, 112, 1613, 10.1007/s00702-005-0378-1 · DOI · Paper page
  78. Waxman, 2009, Molecular mechanisms of α-synuclein neurodegeneration, Biochim Biophys Acta, 1792, 616, 10.1016/j.bbadis.2008.09.013 · DOI
  79. Conway, 1998, Accelerated in vitro fibril formation by a mutant α-synuclein linked to early-onset Parkinson disease, Nat Med, 4, 1318, 10.1038/3311 · DOI
  80. El-Agnaf, 1998, Effects of the mutations Ala30 to Pro and Ala53 to Thr on the physical and morphological properties of α-synuclein protein implicated in Parkinson's disease, FEBS Lett, 440, 67, 10.1016/S0014-5793(98)01419-7 · DOI
  81. Giasson, 1999, Mutant and wild type human α-synucleins assemble into elongated filaments with distinct morphologies in vitro, J Biol Chem, 274, 7619, 10.1074/jbc.274.12.7619 · DOI
  82. Hashimoto, 1998, Human recombinant NACP/α-synuclein is aggregated and fibrillated in vitro: relevance for Lewy body disease, Brain Res, 799, 301, 10.1016/S0006-8993(98)00514-9 · DOI
  83. Narhi, 1999, Both familial Parkinson's disease mutations accelerate α-synuclein aggregation, J Biol Chem, 274, 9843, 10.1074/jbc.274.14.9843 · DOI
  84. Conway, 2000, Fibrils formed in vitro from α-synuclein and two mutant forms linked to Parkinson's disease are typical amyloid, Biochemistry, 39, 2552, 10.1021/bi991447r · DOI
  85. Wood, 1999, α-synuclein fibrillogenesis is nucleation-dependent: implications for the pathogenesis of Parkinson's disease, J Biol Chem, 274, 19509, 10.1074/jbc.274.28.19509 · DOI
  86. Choi, 2004, Mutation E46K increases phospholipid binding and assembly into filaments of human α-synuclein, FEBS Lett, 576, 363, 10.1016/j.febslet.2004.09.038 · DOI
  87. Lee, 2005, Intravesicular localization and exocytosis of α-synuclein and its aggregates, J Neurosci, 25, 6016, 10.1523/JNEUROSCI.0692-05.2005 · DOI
  88. Jang, 2010, Non-classical exocytosis of α-synuclein is sensitive to folding states and promoted under stress conditions, J Neurochem, 113, 1263, 10.1111/j.1471-4159.2010.06695.x · DOI · Paper page
  89. Ahn, 2006, Amino acid sequence motifs and mechanistic features of the membrane translocation of α-synuclein, J Neurochem, 97, 265, 10.1111/j.1471-4159.2006.03731.x · DOI · Paper page
  90. Lee, 2008, Assembly-dependent endocytosis and clearance of extracellular α-synuclein, Int J Biochem Cell Biol, 40, 1835, 10.1016/j.biocel.2008.01.017 · DOI · Paper page
  91. Sung, 2001, Induction of neuronal cell death by Rab5A-dependent endocytosis of α-synuclein, J Biol Chem, 276, 27441, 10.1074/jbc.M101318200 · DOI
  92. Liu, 2009, Rab11a and HSP90 regulate recycling of extracellular α-synuclein, J Neurosci, 29, 1480, 10.1523/JNEUROSCI.6202-08.2009 · DOI
  93. Danzer, 2007, Different species of α-synuclein oligomers induce calcium influx and seeding, J Neurosci, 27, 9220, 10.1523/JNEUROSCI.2617-07.2007 · DOI · Paper page
  94. Danzer, 2009, Seeding induced by α-synuclein oligomers provides evidence for spreading of α-synuclein pathology, J Neurochem, 111, 192, 10.1111/j.1471-4159.2009.06324.x · DOI · Paper page
  95. Luk, 2009, Exogenous α-synuclein fibrils seed the formation of Lewy body-like intracellular inclusions in cultured cells, Proc Natl Acad Sci USA, 106, 20051, 10.1073/pnas.0908005106 · DOI · Paper page
  96. Waxman, 2010, A novel, high-efficiency cellular model of fibrillar α-synuclein inclusions and the examination of mutations that inhibit amyloid formation, J Neurochem, 113, 374, 10.1111/j.1471-4159.2010.06592.x · DOI
  97. Jellinger, 2009, Formation and development of Lewy pathology: a critical update, J Neurol, 256, 270, 10.1007/s00415-009-5243-y · DOI
  98. Gao, 2008, Neuroinflammation and oxidation/nitration of α-synuclein linked to dopaminergic neurodegeneration, J Neurosci, 28, 7687, 10.1523/JNEUROSCI.0143-07.2008 · DOI
  99. Hirsch, 2009, Neuroinflammation in Parkinson's disease: a target for neuroprotection?, Lancet Neurol, 8, 382, 10.1016/S1474-4422(09)70062-6 · DOI · Paper page
  100. Agnati, 2010, A new hypothesis of pathogenesis based on the divorce between mitochondria and their host cells: possible relevance for the Alzheimer's disease, Curr Alzheimer Res, 7, 307, 10.2174/156720510791162395 · DOI
  101. Kuo, 2010, Extensive enteric nervous system abnormalities in mice transgenic for artificial chromosomes containing Parkinson disease-associated α-synuclein gene mutations precede central nervous system changes, Hum Mol Genet, 19, 1633, 10.1093/hmg/ddq038 · DOI
  102. Olanow, 2009, The scientific and clinical basis for the treatment of Parkinson disease, Neurology, 72, S1, 10.1212/WNL.0b013e3181a1d44c · DOI
  103. Olanow, 2009, A double-blind, delayed-start trial of rasagiline in Parkinson's disease, N Engl J Med, 361, 1268, 10.1056/NEJMoa0809335 · DOI
  104. Eller, 2009, Biological fluid biomarkers in neurodegenerative parkinsonism, Nat Rev Neurol, 5, 561, 10.1038/nrneurol.2009.135 · DOI

Cited by

Sorted by citations

Metadata

Authors
Angot, E., Steiner, J. A., Hansen, C., Li, J.-Y., & Brundin, P.
Venue
The Lancet Neurology
Publisher
Elsevier BV
Published
2010-11
Volume
9
Issue
11
Pages
1128-1138
ISSN
1474-4422
DOI
10.1016/s1474-4422(10)70213-1
Cited by
251
References
14
Language
en
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review