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Published in: Acta Neuropathologica 1/2011

Open Access 01-01-2011 | Review

Tau, prions and Aβ: the triad of neurodegeneration

Authors: Lilla Reiniger, Ana Lukic, Jacqueline Linehan, Peter Rudge, John Collinge, Simon Mead, Sebastian Brandner

Published in: Acta Neuropathologica | Issue 1/2011

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Abstract

This article highlights the features that connect prion diseases with other cerebral amyloidoses and how these relate to neurodegeneration, with focus on tau phosphorylation. It also discusses similarities between prion disease and Alzheimer’s disease: mechanisms of amyloid formation, neurotoxicity, pathways involved in triggering tau phosphorylation, links to cell cycle pathways and neuronal apoptosis. We review previous evidence of prion diseases triggering hyperphosphorylation of tau, and complement these findings with cases from our collection of genetic, sporadic and transmitted forms of prion diseases. This includes the novel finding that tau phosphorylation consistently occurs in sporadic CJD, in the absence of amyloid plaques.
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Literature
2.
go back to reference Alafuzoff I, Arzberger T, Al-Sarraj S, Bodi I, Bogdanovic N, Braak H, Bugiani O, Del-Tredici K, Ferrer I, Gelpi E, Giaccone G, Graeber MB, Ince P, Kamphorst W, King A, Korkolopoulou P, Kovacs GG, Larionov S, Meyronet D, Monoranu C, Parchi P, Patsouris E, Roggendorf W, Seilhean D, Tagliavini F, Stadelmann C, Streichenberger N, Thal DR, Wharton SB, Kretzschmar H (2008) Staging of neurofibrillary pathology in Alzheimer’s disease: a study of the BrainNet Europe Consortium. Brain Pathol 18:484–496PubMed Alafuzoff I, Arzberger T, Al-Sarraj S, Bodi I, Bogdanovic N, Braak H, Bugiani O, Del-Tredici K, Ferrer I, Gelpi E, Giaccone G, Graeber MB, Ince P, Kamphorst W, King A, Korkolopoulou P, Kovacs GG, Larionov S, Meyronet D, Monoranu C, Parchi P, Patsouris E, Roggendorf W, Seilhean D, Tagliavini F, Stadelmann C, Streichenberger N, Thal DR, Wharton SB, Kretzschmar H (2008) Staging of neurofibrillary pathology in Alzheimer’s disease: a study of the BrainNet Europe Consortium. Brain Pathol 18:484–496PubMed
3.
go back to reference Alafuzoff I, Pikkarainen M, Al-Sarraj S, Arzberger T, Bell J, Bodi I, Bogdanovic N, Budka H, Bugiani O, Ferrer I, Gelpi E, Giaccone G, Graeber MB, Hauw JJ, Kamphorst W, King A, Kopp N, Korkolopoulou P, Kovacs GG, Meyronet D, Parchi P, Patsouris E, Preusser M, Ravid R, Roggendorf W, Seilhean D, Streichenberger N, Thal DR, Kretzschmar H (2006) Interlaboratory comparison of assessments of Alzheimer disease-related lesions: a study of the BrainNet Europe Consortium. J Neuropathol Exp Neurol 65:740–757CrossRefPubMed Alafuzoff I, Pikkarainen M, Al-Sarraj S, Arzberger T, Bell J, Bodi I, Bogdanovic N, Budka H, Bugiani O, Ferrer I, Gelpi E, Giaccone G, Graeber MB, Hauw JJ, Kamphorst W, King A, Kopp N, Korkolopoulou P, Kovacs GG, Meyronet D, Parchi P, Patsouris E, Preusser M, Ravid R, Roggendorf W, Seilhean D, Streichenberger N, Thal DR, Kretzschmar H (2006) Interlaboratory comparison of assessments of Alzheimer disease-related lesions: a study of the BrainNet Europe Consortium. J Neuropathol Exp Neurol 65:740–757CrossRefPubMed
4.
go back to reference Alafuzoff I, Thal DR, Arzberger T, Bogdanovic N, Al-Sarraj S, Bodi I, Boluda S, Bugiani O, Duyckaerts C, Gelpi E, Gentleman S, Giaccone G, Graeber M, Hortobagyi T, Hoftberger R, Ince P, Ironside JW, Kavantzas N, King A, Korkolopoulou P, Kovacs GG, Meyronet D, Monoranu C, Nilsson T, Parchi P, Patsouris E, Pikkarainen M, Revesz T, Rozemuller A, Seilhean D, Schulz-Schaeffer W, Streichenberger N, Wharton SB, Kretzschmar H (2009) Assessment of beta-amyloid deposits in human brain: a study of the BrainNet Europe Consortium. Acta Neuropathol 117:309–320CrossRefPubMed Alafuzoff I, Thal DR, Arzberger T, Bogdanovic N, Al-Sarraj S, Bodi I, Boluda S, Bugiani O, Duyckaerts C, Gelpi E, Gentleman S, Giaccone G, Graeber M, Hortobagyi T, Hoftberger R, Ince P, Ironside JW, Kavantzas N, King A, Korkolopoulou P, Kovacs GG, Meyronet D, Monoranu C, Nilsson T, Parchi P, Patsouris E, Pikkarainen M, Revesz T, Rozemuller A, Seilhean D, Schulz-Schaeffer W, Streichenberger N, Wharton SB, Kretzschmar H (2009) Assessment of beta-amyloid deposits in human brain: a study of the BrainNet Europe Consortium. Acta Neuropathol 117:309–320CrossRefPubMed
5.
go back to reference Amano N, Yagishita S, Yokoi S, Itoh Y, Kinoshita J, Mizutani T, Matsuishi T (1992) Gerstmann–Straussler syndrome–a variant type: amyloid plaques and Alzheimer’s neurofibrillary tangles in cerebral cortex. Acta Neuropathol Berl 84:15–23CrossRefPubMed Amano N, Yagishita S, Yokoi S, Itoh Y, Kinoshita J, Mizutani T, Matsuishi T (1992) Gerstmann–Straussler syndrome–a variant type: amyloid plaques and Alzheimer’s neurofibrillary tangles in cerebral cortex. Acta Neuropathol Berl 84:15–23CrossRefPubMed
6.
go back to reference Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood AL, Welch J, Hill AF, Lloyd SE, Wadsworth JD, Collinge J (2002) BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 21:6358–6366 Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood AL, Welch J, Hill AF, Lloyd SE, Wadsworth JD, Collinge J (2002) BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 21:6358–6366
7.
go back to reference Bitan G, Fradinger EA, Spring SM, Teplow DB (2005) Neurotoxic protein oligomers–what you see is not always what you get. Amyloid 12:88–95CrossRefPubMed Bitan G, Fradinger EA, Spring SM, Teplow DB (2005) Neurotoxic protein oligomers–what you see is not always what you get. Amyloid 12:88–95CrossRefPubMed
8.
go back to reference Boellaard JW, Doerr Schott J, Schlote W (1993) Miniplaques and shapeless cerebral amyloid deposits in a case of Gerstmann–Straussler–Scheinker’s syndrome. Acta Neuropathol Berl 86:532–535CrossRefPubMed Boellaard JW, Doerr Schott J, Schlote W (1993) Miniplaques and shapeless cerebral amyloid deposits in a case of Gerstmann–Straussler–Scheinker’s syndrome. Acta Neuropathol Berl 86:532–535CrossRefPubMed
9.
go back to reference Bolmont T, Clavaguera F, Meyer-Luehmann M, Herzig MC, Radde R, Staufenbiel M, Lewis J, Hutton M, Tolnay M, Jucker M (2007) Induction of tau pathology by intracerebral infusion of amyloid-beta-containing brain extract and by amyloid-beta deposition in APP × Tau transgenic mice. Am J Pathol 171:2012–2020CrossRefPubMed Bolmont T, Clavaguera F, Meyer-Luehmann M, Herzig MC, Radde R, Staufenbiel M, Lewis J, Hutton M, Tolnay M, Jucker M (2007) Induction of tau pathology by intracerebral infusion of amyloid-beta-containing brain extract and by amyloid-beta deposition in APP × Tau transgenic mice. Am J Pathol 171:2012–2020CrossRefPubMed
10.
go back to reference Braak H, Braak E (1995) Staging of Alzheimer’s disease-related neurofibrillary changes. Neurobiol Aging 16:271–278 (discussion 278–284)CrossRefPubMed Braak H, Braak E (1995) Staging of Alzheimer’s disease-related neurofibrillary changes. Neurobiol Aging 16:271–278 (discussion 278–284)CrossRefPubMed
11.
go back to reference Braak H, Braak E, Bohl J (1993) Staging of Alzheimer-related cortical destruction. Eur Neurol 33:403–408CrossRefPubMed Braak H, Braak E, Bohl J (1993) Staging of Alzheimer-related cortical destruction. Eur Neurol 33:403–408CrossRefPubMed
12.
go back to reference Brandner S, Whitfield J, Boone K, Puwa A, O’Malley C, Linehan JM, Joiner S, Scaravilli F, Calder I, PA M, Wadsworth JD, Collinge J (2008) Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease. Philos Trans R Soc Lond B Biol Sci 363:3755–3763CrossRefPubMed Brandner S, Whitfield J, Boone K, Puwa A, O’Malley C, Linehan JM, Joiner S, Scaravilli F, Calder I, PA M, Wadsworth JD, Collinge J (2008) Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease. Philos Trans R Soc Lond B Biol Sci 363:3755–3763CrossRefPubMed
13.
go back to reference Brown DR (2000) Altered toxicity of the prion protein peptide PrP106–126 carrying the Ala(117)>Val mutation. Biochem J 346(Pt 3):785–791CrossRefPubMed Brown DR (2000) Altered toxicity of the prion protein peptide PrP106–126 carrying the Ala(117)>Val mutation. Biochem J 346(Pt 3):785–791CrossRefPubMed
14.
go back to reference Brown P, Preece M, Brandel JP, Sato T, McShane L, Zerr I, Fletcher A, Will RG, Pocchiari M, Cashman NR, d’Aignaux JH, Cervenakova L, Fradkin J, Schonberger LB, Collins SJ (2000) Iatrogenic Creutzfeldt–Jakob disease at the millennium. Neurology 55:1075–1081PubMed Brown P, Preece M, Brandel JP, Sato T, McShane L, Zerr I, Fletcher A, Will RG, Pocchiari M, Cashman NR, d’Aignaux JH, Cervenakova L, Fradkin J, Schonberger LB, Collins SJ (2000) Iatrogenic Creutzfeldt–Jakob disease at the millennium. Neurology 55:1075–1081PubMed
15.
go back to reference Brown P, Preece MA, Will RG (1992) “Friendly fire” in medicine: hormones, homografts, and Creutzfeldt–Jakob disease. Lancet 340:24–27CrossRefPubMed Brown P, Preece MA, Will RG (1992) “Friendly fire” in medicine: hormones, homografts, and Creutzfeldt–Jakob disease. Lancet 340:24–27CrossRefPubMed
16.
go back to reference Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock CJ (1997) Transmissions to mice indicate that ‘new variant' CJD is caused by the BSE agent. Nature 389:498–501 Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock CJ (1997) Transmissions to mice indicate that ‘new variant' CJD is caused by the BSE agent. Nature 389:498–501
17.
go back to reference Bu B, Klunemann H, Suzuki K, Li J, Bird T, Jin LW, Vincent I (2002) Niemann–Pick disease type C yields possible clue for why cerebellar neurons do not form neurofibrillary tangles. Neurobiol Dis 11:285–297CrossRefPubMed Bu B, Klunemann H, Suzuki K, Li J, Bird T, Jin LW, Vincent I (2002) Niemann–Pick disease type C yields possible clue for why cerebellar neurons do not form neurofibrillary tangles. Neurobiol Dis 11:285–297CrossRefPubMed
18.
go back to reference Bu B, Li J, Davies P, Vincent I (2002) Deregulation of cdk5, hyperphosphorylation, and cytoskeletal pathology in the Niemann–Pick type C murine model. J Neurosci 22:6515–6525PubMed Bu B, Li J, Davies P, Vincent I (2002) Deregulation of cdk5, hyperphosphorylation, and cytoskeletal pathology in the Niemann–Pick type C murine model. J Neurosci 22:6515–6525PubMed
19.
go back to reference Cadavid D, Mena H, Koeller K, Frommelt RA (2000) Cerebral beta amyloid angiopathy is a risk factor for cerebral ischemic infarction: a case control study in human brain biopsies. J Neuropathol Exp Neurol 59:768–773PubMed Cadavid D, Mena H, Koeller K, Frommelt RA (2000) Cerebral beta amyloid angiopathy is a risk factor for cerebral ischemic infarction: a case control study in human brain biopsies. J Neuropathol Exp Neurol 59:768–773PubMed
20.
go back to reference Camins A, Verdaguer E, Folch J, Canudas AM, Pallas M (2006) The role of CDK5/P25 formation/inhibition in neurodegeneration. Drug News Perspect 19:453–460CrossRefPubMed Camins A, Verdaguer E, Folch J, Canudas AM, Pallas M (2006) The role of CDK5/P25 formation/inhibition in neurodegeneration. Drug News Perspect 19:453–460CrossRefPubMed
21.
go back to reference Campbell TA, Palmer MS, Will RG, Gibb WR, Luthert PJ, Collinge J (1996) A prion disease with a novel 96-base pair insertional mutation in the prion protein gene. Neurology 46:761–766PubMed Campbell TA, Palmer MS, Will RG, Gibb WR, Luthert PJ, Collinge J (1996) A prion disease with a novel 96-base pair insertional mutation in the prion protein gene. Neurology 46:761–766PubMed
22.
go back to reference Capellari S, Vital C, Parchi P, Petersen RB, Ferrer X, Jarnier D, Pegoraro E, Gambetti P, Julien J (1997) Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family. Neurology 49:133–141PubMed Capellari S, Vital C, Parchi P, Petersen RB, Ferrer X, Jarnier D, Pegoraro E, Gambetti P, Julien J (1997) Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family. Neurology 49:133–141PubMed
23.
go back to reference Clavaguera F, Bolmont T, Crowther RA, Abramowski D, Frank S, Probst A, Fraser G, Stalder AK, Beibel M, Staufenbiel M, Jucker M, Goedert M, Tolnay M (2009) Transmission and spreading of tauopathy in transgenic mouse brain. Nat Cell Biol 11:909–913CrossRefPubMed Clavaguera F, Bolmont T, Crowther RA, Abramowski D, Frank S, Probst A, Fraser G, Stalder AK, Beibel M, Staufenbiel M, Jucker M, Goedert M, Tolnay M (2009) Transmission and spreading of tauopathy in transgenic mouse brain. Nat Cell Biol 11:909–913CrossRefPubMed
24.
go back to reference Collinge J, Clarke AR (2007) A general model of prion strains and their pathogenicity. Science 318:930–936CrossRefPubMed Collinge J, Clarke AR (2007) A general model of prion strains and their pathogenicity. Science 318:930–936CrossRefPubMed
25.
go back to reference Collinge J, Sidle KC, Meads J, Ironside J, Hill AF (1996) Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 383:685–690 Collinge J, Sidle KC, Meads J, Ironside J, Hill AF (1996) Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 383:685–690
26.
go back to reference Cuny E, Loiseau H, Rivel J, Vital C, Castel JP (1996) Amyloid angiopathy-related cerebellar hemorrhage. Surg Neurol 46:235–239CrossRefPubMed Cuny E, Loiseau H, Rivel J, Vital C, Castel JP (1996) Amyloid angiopathy-related cerebellar hemorrhage. Surg Neurol 46:235–239CrossRefPubMed
27.
go back to reference Duchen LW, Poulter M, Harding AE (1993) Dementia associated with a 216 base pair insertion in the prion protein gene. Clinical and neuropathological features. Brain 116:555–567CrossRefPubMed Duchen LW, Poulter M, Harding AE (1993) Dementia associated with a 216 base pair insertion in the prion protein gene. Clinical and neuropathological features. Brain 116:555–567CrossRefPubMed
28.
go back to reference Duffy P, Wolf J, Collins G, DeVoe AG, Streeten B, Cowen D (1974) Possible person-to-person transmission of Creutzfeldt–Jakob disease. N Engl J Med 290:692–693PubMed Duffy P, Wolf J, Collins G, DeVoe AG, Streeten B, Cowen D (1974) Possible person-to-person transmission of Creutzfeldt–Jakob disease. N Engl J Med 290:692–693PubMed
29.
go back to reference Eisele YS, Bolmont T, Heikenwalder M, Langer F, Jacobson LH, Yan ZX, Roth K, Aguzzi A, Staufenbiel M, Walker LC, Jucker M (2009) Induction of cerebral beta-amyloidosis: intracerebral versus systemic Abeta inoculation. Proc Natl Acad Sci USA 106:12926–12931CrossRefPubMed Eisele YS, Bolmont T, Heikenwalder M, Langer F, Jacobson LH, Yan ZX, Roth K, Aguzzi A, Staufenbiel M, Walker LC, Jucker M (2009) Induction of cerebral beta-amyloidosis: intracerebral versus systemic Abeta inoculation. Proc Natl Acad Sci USA 106:12926–12931CrossRefPubMed
30.
go back to reference Fein JA, Sokolow S, Miller CA, Vinters HV, Yang F, Cole GM, Gylys KH (2008) Co-localization of amyloid beta and tau pathology in Alzheimer’s disease synaptosomes. Am J Pathol 172:1683–1692CrossRefPubMed Fein JA, Sokolow S, Miller CA, Vinters HV, Yang F, Cole GM, Gylys KH (2008) Co-localization of amyloid beta and tau pathology in Alzheimer’s disease synaptosomes. Am J Pathol 172:1683–1692CrossRefPubMed
31.
32.
go back to reference Fukutani Y, Cairns NJ, Rossor MN, Lantos PL (1997) Cerebellar pathology in sporadic and familial Alzheimer’s disease including APP 717 (Val > Ile) mutation cases: a morphometric investigation. J Neurol Sci 149:177–184CrossRefPubMed Fukutani Y, Cairns NJ, Rossor MN, Lantos PL (1997) Cerebellar pathology in sporadic and familial Alzheimer’s disease including APP 717 (Val > Ile) mutation cases: a morphometric investigation. J Neurol Sci 149:177–184CrossRefPubMed
33.
go back to reference Ghetti B, Piccardo P, Spillantini MG, Ichimiya Y, Porro M, Perini F, Kitamoto T, Tateishi J, Seiler C, Frangione B, Bugiani O, Giaccone G, Prelli F, Goedert M, Dlouhy SR, Tagliavini F (1996) Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc Natl Acad Sci USA 93:744–748CrossRefPubMed Ghetti B, Piccardo P, Spillantini MG, Ichimiya Y, Porro M, Perini F, Kitamoto T, Tateishi J, Seiler C, Frangione B, Bugiani O, Giaccone G, Prelli F, Goedert M, Dlouhy SR, Tagliavini F (1996) Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc Natl Acad Sci USA 93:744–748CrossRefPubMed
34.
go back to reference Ghetti B, Tagliavini F, Giaccone G, Bugiani O, Frangione B, Farlow MR, Dlouhy SR (1994) Familial Gerstmann–Straussler–Scheinker disease with neurofibrillary tangles. Mol Neurobiol 8:41–48CrossRefPubMed Ghetti B, Tagliavini F, Giaccone G, Bugiani O, Frangione B, Farlow MR, Dlouhy SR (1994) Familial Gerstmann–Straussler–Scheinker disease with neurofibrillary tangles. Mol Neurobiol 8:41–48CrossRefPubMed
35.
go back to reference Ghetti B, Tagliavini F, Masters CL, Beyreuther K, Giaccone G, Verga L, Farlow MR, Conneally PM, Dlouhy SR, Azzarelli B et al (1989) Gerstmann–Straussler–Scheinker disease, II: Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family. Neurology 39:1453–1461PubMed Ghetti B, Tagliavini F, Masters CL, Beyreuther K, Giaccone G, Verga L, Farlow MR, Conneally PM, Dlouhy SR, Azzarelli B et al (1989) Gerstmann–Straussler–Scheinker disease, II: Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family. Neurology 39:1453–1461PubMed
36.
go back to reference Giaccone G, Mangieri M, Capobianco R, Limido L, Hauw JJ, Haik S, Fociani P, Bugiani O, Tagliavini F (2008) Tauopathy in human and experimental variant Creutzfeldt–Jakob disease. Neurobiol Aging 29:1864–1873CrossRefPubMed Giaccone G, Mangieri M, Capobianco R, Limido L, Hauw JJ, Haik S, Fociani P, Bugiani O, Tagliavini F (2008) Tauopathy in human and experimental variant Creutzfeldt–Jakob disease. Neurobiol Aging 29:1864–1873CrossRefPubMed
37.
go back to reference Glabe C (2001) Intracellular mechanisms of amyloid accumulation and pathogenesis in Alzheimer’s disease. J Mol Neurosci 17:137–145CrossRefPubMed Glabe C (2001) Intracellular mechanisms of amyloid accumulation and pathogenesis in Alzheimer’s disease. J Mol Neurosci 17:137–145CrossRefPubMed
38.
go back to reference Gotz J, Chen F, van Dorpe J, Nitsch RM (2001) Formation of neurofibrillary tangles in P301l tau transgenic mice induced by Abeta 42 fibrils. Science 293:1491–1495CrossRefPubMed Gotz J, Chen F, van Dorpe J, Nitsch RM (2001) Formation of neurofibrillary tangles in P301l tau transgenic mice induced by Abeta 42 fibrils. Science 293:1491–1495CrossRefPubMed
39.
go back to reference Griffin JP (1991) Transmission of Creutzfeldt–Jakob disease by investigative and therapeutic procedures. Adverse Drug React Toxicol Rev 10:89–98PubMed Griffin JP (1991) Transmission of Creutzfeldt–Jakob disease by investigative and therapeutic procedures. Adverse Drug React Toxicol Rev 10:89–98PubMed
41.
go back to reference Gunther EC, Strittmatter SM (2010) beta-Amyloid oligomers and cellular prion protein in Alzheimer’s disease. J Mol Med 88:331–338CrossRefPubMed Gunther EC, Strittmatter SM (2010) beta-Amyloid oligomers and cellular prion protein in Alzheimer’s disease. J Mol Med 88:331–338CrossRefPubMed
42.
go back to reference Haass C, Selkoe DJ (2007) Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer’s amyloid beta-peptide. Nat Rev Mol Cell Biol 8:101–112CrossRefPubMed Haass C, Selkoe DJ (2007) Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer’s amyloid beta-peptide. Nat Rev Mol Cell Biol 8:101–112CrossRefPubMed
43.
go back to reference Hainfellner JA, Parchi P, Kitamoto T, Jarius C, Gambetti P, Budka H (1999) A novel phenotype in familial Creutzfeldt–Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein. Ann Neurol 45:812–816CrossRefPubMed Hainfellner JA, Parchi P, Kitamoto T, Jarius C, Gambetti P, Budka H (1999) A novel phenotype in familial Creutzfeldt–Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein. Ann Neurol 45:812–816CrossRefPubMed
44.
go back to reference Hardy JA, Higgins GA (1992) Alzheimer’s disease: the amyloid cascade hypothesis. Science 256:184–185CrossRefPubMed Hardy JA, Higgins GA (1992) Alzheimer’s disease: the amyloid cascade hypothesis. Science 256:184–185CrossRefPubMed
45.
go back to reference Hernandez F, de Barreda EG, Fuster-Matanzo A, Goni-Oliver P, Lucas JJ, Avila J (2009) The role of GSK3 in Alzheimer disease. Brain Res Bull 80:248–250CrossRefPubMed Hernandez F, de Barreda EG, Fuster-Matanzo A, Goni-Oliver P, Lucas JJ, Avila J (2009) The role of GSK3 in Alzheimer disease. Brain Res Bull 80:248–250CrossRefPubMed
47.
go back to reference Herzberg L (1979) Creutzfeld–Jakob disease and corneal grafts. Med J Aust 1:248PubMed Herzberg L (1979) Creutzfeld–Jakob disease and corneal grafts. Med J Aust 1:248PubMed
48.
go back to reference Herzig MC, Winkler DT, Burgermeister P, Pfeifer M, Kohler E, Schmidt SD, Danner S, Abramowski D, Sturchler-Pierrat C, Burki K, van Duinen SG, Maat-Schieman ML, Staufenbiel M, Mathews PM, Jucker M (2004) Abeta is targeted to the vasculature in a mouse model of hereditary cerebral hemorrhage with amyloidosis. Nat Neurosci 7:954–960CrossRefPubMed Herzig MC, Winkler DT, Burgermeister P, Pfeifer M, Kohler E, Schmidt SD, Danner S, Abramowski D, Sturchler-Pierrat C, Burki K, van Duinen SG, Maat-Schieman ML, Staufenbiel M, Mathews PM, Jucker M (2004) Abeta is targeted to the vasculature in a mouse model of hereditary cerebral hemorrhage with amyloidosis. Nat Neurosci 7:954–960CrossRefPubMed
49.
go back to reference Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P (1997) The same prion strain causes vCJD and BSE. Nature 389:448–450, 526 Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P (1997) The same prion strain causes vCJD and BSE. Nature 389:448–450, 526
50.
go back to reference Holton JL, Ghiso J, Lashley T, Rostagno A, Guerin CJ, Gibb G, Houlden H, Ayling H, Martinian L, Anderton BH, Wood NW, Vidal R, Plant G, Frangione B, Revesz T (2001) Regional distribution of amyloid-Bri deposition and its association with neurofibrillary degeneration in familial British dementia. Am J Pathol 158:515–526PubMed Holton JL, Ghiso J, Lashley T, Rostagno A, Guerin CJ, Gibb G, Houlden H, Ayling H, Martinian L, Anderton BH, Wood NW, Vidal R, Plant G, Frangione B, Revesz T (2001) Regional distribution of amyloid-Bri deposition and its association with neurofibrillary degeneration in familial British dementia. Am J Pathol 158:515–526PubMed
51.
go back to reference Hooper C, Killick R, Lovestone S (2008) The GSK3 hypothesis of Alzheimer’s disease. J Neurochem 104:1433–1439CrossRefPubMed Hooper C, Killick R, Lovestone S (2008) The GSK3 hypothesis of Alzheimer’s disease. J Neurochem 104:1433–1439CrossRefPubMed
52.
go back to reference Ishizawa K, Komori T, Shimazu T, Yamamoto T, Kitamoto T, Shimazu K, Hirose T (2002) Hyperphosphorylated tau deposition parallels prion protein burden in a case of Gerstmann–Straussler–Scheinker syndrome P102L mutation complicated with dementia. Acta Neuropathol 104:342–350PubMed Ishizawa K, Komori T, Shimazu T, Yamamoto T, Kitamoto T, Shimazu K, Hirose T (2002) Hyperphosphorylated tau deposition parallels prion protein burden in a case of Gerstmann–Straussler–Scheinker syndrome P102L mutation complicated with dementia. Acta Neuropathol 104:342–350PubMed
53.
go back to reference Itoh Y, Yamada M, Hayakawa M, Shozawa T, Tanaka J, Matsushita M, Kitamoto T, Tateishi J, Otomo E (1994) A variant of Gerstmann–Straussler–Scheinker disease carrying codon 105 mutation with codon 129 polymorphism of the prion protein gene: a clinicopathological study. J Neurol Sci 127:77–86CrossRefPubMed Itoh Y, Yamada M, Hayakawa M, Shozawa T, Tanaka J, Matsushita M, Kitamoto T, Tateishi J, Otomo E (1994) A variant of Gerstmann–Straussler–Scheinker disease carrying codon 105 mutation with codon 129 polymorphism of the prion protein gene: a clinicopathological study. J Neurol Sci 127:77–86CrossRefPubMed
54.
go back to reference Kalaria RN, Cohen DL, Greenberg BD, Savage MJ, Bogdanovic NE, Winblad B, Lannfelt L, Adem A (1996) Abundance of the longer A beta 42 in neocortical and cerebrovascular amyloid beta deposits in Swedish familial Alzheimer’s disease and Down’s syndrome. Neuroreport 7:1377–1381CrossRefPubMed Kalaria RN, Cohen DL, Greenberg BD, Savage MJ, Bogdanovic NE, Winblad B, Lannfelt L, Adem A (1996) Abundance of the longer A beta 42 in neocortical and cerebrovascular amyloid beta deposits in Swedish familial Alzheimer’s disease and Down’s syndrome. Neuroreport 7:1377–1381CrossRefPubMed
55.
go back to reference Kanazawa M, Shimohata T, Toyoshima Y, Tada M, Kakita A, Morita T, Ozawa T, Takahashi H, Nishizawa M (2009) Cerebellar involvement in progressive supranuclear palsy: a clinicopathological study. Mov Disord 24:1312–1318CrossRefPubMed Kanazawa M, Shimohata T, Toyoshima Y, Tada M, Kakita A, Morita T, Ozawa T, Takahashi H, Nishizawa M (2009) Cerebellar involvement in progressive supranuclear palsy: a clinicopathological study. Mov Disord 24:1312–1318CrossRefPubMed
56.
go back to reference Kerr F, Rickle A, Nayeem N, Brandner S, Cowburn RF, Lovestone S (2006) PTEN, a negative regulator of PI3 kinase signalling, alters tau phosphorylation in cells by mechanisms independent of GSK-3. FEBS Lett 580:3121–3128CrossRefPubMed Kerr F, Rickle A, Nayeem N, Brandner S, Cowburn RF, Lovestone S (2006) PTEN, a negative regulator of PI3 kinase signalling, alters tau phosphorylation in cells by mechanisms independent of GSK-3. FEBS Lett 580:3121–3128CrossRefPubMed
57.
go back to reference Krasnianski A, von Ahsen N, Heinemann U, Meissner B, Kretzschmar HA, Armstrong VW, Zerr I (2008) ApoE distribution and family history in genetic prion diseases in Germany. J Mol Neurosci 34:45–50CrossRefPubMed Krasnianski A, von Ahsen N, Heinemann U, Meissner B, Kretzschmar HA, Armstrong VW, Zerr I (2008) ApoE distribution and family history in genetic prion diseases in Germany. J Mol Neurosci 34:45–50CrossRefPubMed
58.
59.
go back to reference Lauren J, Gimbel DA, Nygaard HB, Gilbert JW, Strittmatter SM (2009) Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature 457:1128–1132CrossRefPubMed Lauren J, Gimbel DA, Nygaard HB, Gilbert JW, Strittmatter SM (2009) Cellular prion protein mediates impairment of synaptic plasticity by amyloid-beta oligomers. Nature 457:1128–1132CrossRefPubMed
60.
go back to reference Lebel M, Patenaude C, Allyson J, Massicotte G, Cyr M (2009) Dopamine D1 receptor activation induces tau phosphorylation via cdk5 and GSK3 signaling pathways. Neuropharmacology 57:392–402CrossRefPubMed Lebel M, Patenaude C, Allyson J, Massicotte G, Cyr M (2009) Dopamine D1 receptor activation induces tau phosphorylation via cdk5 and GSK3 signaling pathways. Neuropharmacology 57:392–402CrossRefPubMed
61.
go back to reference Lesne S, Koh MT, Kotilinek L, Kayed R, Glabe CG, Yang A, Gallagher M, Ashe KH (2006) A specific amyloid-beta protein assembly in the brain impairs memory. Nature 440:352–357CrossRefPubMed Lesne S, Koh MT, Kotilinek L, Kayed R, Glabe CG, Yang A, Gallagher M, Ashe KH (2006) A specific amyloid-beta protein assembly in the brain impairs memory. Nature 440:352–357CrossRefPubMed
62.
go back to reference Lewis J, Dickson DW, Lin WL, Chisholm L, Corral A, Jones G, Yen SH, Sahara N, Skipper L, Yager D, Eckman C, Hardy J, Hutton M, McGowan E (2001) Enhanced neurofibrillary degeneration in transgenic mice expressing mutant tau and APP. Science 293:1487–1491CrossRefPubMed Lewis J, Dickson DW, Lin WL, Chisholm L, Corral A, Jones G, Yen SH, Sahara N, Skipper L, Yager D, Eckman C, Hardy J, Hutton M, McGowan E (2001) Enhanced neurofibrillary degeneration in transgenic mice expressing mutant tau and APP. Science 293:1487–1491CrossRefPubMed
63.
go back to reference Lewis J, McGowan E, Rockwood J, Melrose H, Nacharaju P, Van Slegtenhorst M, Gwinn-Hardy K, Paul Murphy M, Baker M, Yu X, Duff K, Hardy J, Corral A, Lin WL, Yen SH, Dickson DW, Davies P, Hutton M (2000) Neurofibrillary tangles, amyotrophy and progressive motor disturbance in mice expressing mutant (P301L) tau protein. Nat Genet 25:402–405CrossRefPubMed Lewis J, McGowan E, Rockwood J, Melrose H, Nacharaju P, Van Slegtenhorst M, Gwinn-Hardy K, Paul Murphy M, Baker M, Yu X, Duff K, Hardy J, Corral A, Lin WL, Yen SH, Dickson DW, Davies P, Hutton M (2000) Neurofibrillary tangles, amyotrophy and progressive motor disturbance in mice expressing mutant (P301L) tau protein. Nat Genet 25:402–405CrossRefPubMed
64.
go back to reference Lopes JP, Oliveira CR, Agostinho P (2007) Role of cyclin-dependent kinase 5 in the neurodegenerative process triggered by amyloid-Beta and prion peptides: implications for Alzheimer’s disease and prion-related encephalopathies. Cell Mol Neurobiol 27:943–957CrossRefPubMed Lopes JP, Oliveira CR, Agostinho P (2007) Role of cyclin-dependent kinase 5 in the neurodegenerative process triggered by amyloid-Beta and prion peptides: implications for Alzheimer’s disease and prion-related encephalopathies. Cell Mol Neurobiol 27:943–957CrossRefPubMed
65.
go back to reference Lopes JP, Oliveira CR, Agostinho P (2009) Cdk5 acts as a mediator of neuronal cell cycle re-entry triggered by amyloid-beta and prion peptides. Cell Cycle 8:97–104PubMed Lopes JP, Oliveira CR, Agostinho P (2009) Cdk5 acts as a mediator of neuronal cell cycle re-entry triggered by amyloid-beta and prion peptides. Cell Cycle 8:97–104PubMed
66.
go back to reference Love S, Bridges LR, Case CP (1995) Neurofibrillary tangles in Niemann–Pick disease type C. Brain 118(Pt 1):119–129CrossRefPubMed Love S, Bridges LR, Case CP (1995) Neurofibrillary tangles in Niemann–Pick disease type C. Brain 118(Pt 1):119–129CrossRefPubMed
67.
go back to reference Marino S, Krimpenfort P, Leung C, van der Korput HA, Trapman J, Camenisch I, Berns A, Brandner S (2002) PTEN is essential for cell migration but not for fate determination and tumourigenesis in the cerebellum. Development 129:3513–3522PubMed Marino S, Krimpenfort P, Leung C, van der Korput HA, Trapman J, Camenisch I, Berns A, Brandner S (2002) PTEN is essential for cell migration but not for fate determination and tumourigenesis in the cerebellum. Development 129:3513–3522PubMed
68.
go back to reference Mead S, James-Galton M, Revesz T, Doshi RB, Harwood G, Pan EL, Ghiso J, Frangione B, Plant G (2000) Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings. Brain 123(Pt 5):975–991CrossRefPubMed Mead S, James-Galton M, Revesz T, Doshi RB, Harwood G, Pan EL, Ghiso J, Frangione B, Plant G (2000) Familial British dementia with amyloid angiopathy: early clinical, neuropsychological and imaging findings. Brain 123(Pt 5):975–991CrossRefPubMed
69.
go back to reference 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, Staufenbiel M, Walker LC, Jucker M (2006) Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science 313:1781–1784CrossRefPubMed 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, Staufenbiel M, Walker LC, Jucker M (2006) Exogenous induction of cerebral beta-amyloidogenesis is governed by agent and host. Science 313:1781–1784CrossRefPubMed
70.
go back to reference Mori H, Takio K, Ogawara M, Selkoe DJ (1992) Mass spectrometry of purified amyloid beta protein in Alzheimer’s disease. J Biol Chem 267:17082–17086PubMed Mori H, Takio K, Ogawara M, Selkoe DJ (1992) Mass spectrometry of purified amyloid beta protein in Alzheimer’s disease. J Biol Chem 267:17082–17086PubMed
71.
go back to reference Nayeem N, Kerr F, Naumann H, Linehan J, Lovestone S, Brandner S (2007) Hyperphosphorylation of tau and neurofilaments and activation of CDK5 and ERK1/2 in PTEN-deficient cerebella. Mol Cell Neurosci 34:400–408CrossRefPubMed Nayeem N, Kerr F, Naumann H, Linehan J, Lovestone S, Brandner S (2007) Hyperphosphorylation of tau and neurofilaments and activation of CDK5 and ERK1/2 in PTEN-deficient cerebella. Mol Cell Neurosci 34:400–408CrossRefPubMed
72.
go back to reference Nishitsuji K, Tomiyama T, Ishibashi K, Kametani F, Ozawa K, Okada R, Maat-Schieman ML, Roos RA, Iwai K, Mori H (2007) Cerebral vascular accumulation of Dutch-type Abeta42, but not wild-type Abeta42, in hereditary cerebral hemorrhage with amyloidosis, Dutch type. J Neurosci Res 85:2917–2923CrossRefPubMed Nishitsuji K, Tomiyama T, Ishibashi K, Kametani F, Ozawa K, Okada R, Maat-Schieman ML, Roos RA, Iwai K, Mori H (2007) Cerebral vascular accumulation of Dutch-type Abeta42, but not wild-type Abeta42, in hereditary cerebral hemorrhage with amyloidosis, Dutch type. J Neurosci Res 85:2917–2923CrossRefPubMed
73.
go back to reference Nochlin D, Sumi SM, Bird TD, Snow AD, Leventhal CM, Beyreuther K, Masters CL (1989) Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann–Straussler syndrome. Neurology 39:910–918PubMed Nochlin D, Sumi SM, Bird TD, Snow AD, Leventhal CM, Beyreuther K, Masters CL (1989) Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann–Straussler syndrome. Neurology 39:910–918PubMed
74.
go back to reference Nygaard HB, Strittmatter SM (2009) Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease. Arch Neurol 66:1325–1328CrossRefPubMed Nygaard HB, Strittmatter SM (2009) Cellular prion protein mediates the toxicity of beta-amyloid oligomers: implications for Alzheimer disease. Arch Neurol 66:1325–1328CrossRefPubMed
75.
go back to reference Oddo S, Billings L, Kesslak JP, Cribbs DH, LaFerla FM (2004) Abeta immunotherapy leads to clearance of early, but not late, hyperphosphorylated tau aggregates via the proteasome. Neuron 43:321–332CrossRefPubMed Oddo S, Billings L, Kesslak JP, Cribbs DH, LaFerla FM (2004) Abeta immunotherapy leads to clearance of early, but not late, hyperphosphorylated tau aggregates via the proteasome. Neuron 43:321–332CrossRefPubMed
76.
go back to reference Okawa Y, Ishiguro K, Fujita SC (2003) Stress-induced hyperphosphorylation of tau in the mouse brain. FEBS Lett 535:183–189CrossRefPubMed Okawa Y, Ishiguro K, Fujita SC (2003) Stress-induced hyperphosphorylation of tau in the mouse brain. FEBS Lett 535:183–189CrossRefPubMed
77.
go back to reference Parkin ET, Watt NT, Hussain I, Eckman EA, Eckman CB, Manson JC, Baybutt HN, Turner AJ, Hooper NM (2007) Cellular prion protein regulates beta-secretase cleavage of the Alzheimer’s amyloid precursor protein. Proc Natl Acad Sci USA 104:11062–11067CrossRefPubMed Parkin ET, Watt NT, Hussain I, Eckman EA, Eckman CB, Manson JC, Baybutt HN, Turner AJ, Hooper NM (2007) Cellular prion protein regulates beta-secretase cleavage of the Alzheimer’s amyloid precursor protein. Proc Natl Acad Sci USA 104:11062–11067CrossRefPubMed
78.
go back to reference Patrick GN, Zukerberg L, Nikolic M, de la Monte S, Dikkes P, Tsai LH (1999) Conversion of p35 to p25 deregulates Cdk5 activity and promotes neurodegeneration. Nature 402:615–622CrossRefPubMed Patrick GN, Zukerberg L, Nikolic M, de la Monte S, Dikkes P, Tsai LH (1999) Conversion of p35 to p25 deregulates Cdk5 activity and promotes neurodegeneration. Nature 402:615–622CrossRefPubMed
79.
go back to reference Perez M, Rojo AI, Wandosell F, Diaz-Nido J, Avila J (2003) Prion peptide induces neuronal cell death through a pathway involving glycogen synthase kinase 3. Biochem J 372:129–136CrossRefPubMed Perez M, Rojo AI, Wandosell F, Diaz-Nido J, Avila J (2003) Prion peptide induces neuronal cell death through a pathway involving glycogen synthase kinase 3. Biochem J 372:129–136CrossRefPubMed
80.
go back to reference Piao YS, Hayashi S, Wakabayashi K, Kakita A, Aida I, Yamada M, Takahashi H (2002) Cerebellar cortical tau pathology in progressive supranuclear palsy and corticobasal degeneration. Acta Neuropathol 103:469–474CrossRefPubMed Piao YS, Hayashi S, Wakabayashi K, Kakita A, Aida I, Yamada M, Takahashi H (2002) Cerebellar cortical tau pathology in progressive supranuclear palsy and corticobasal degeneration. Acta Neuropathol 103:469–474CrossRefPubMed
81.
go back to reference Pimplikar SW (2009) Reassessing the amyloid cascade hypothesis of Alzheimer’s disease. Int J Biochem Cell Biol 41:1261–1268CrossRefPubMed Pimplikar SW (2009) Reassessing the amyloid cascade hypothesis of Alzheimer’s disease. Int J Biochem Cell Biol 41:1261–1268CrossRefPubMed
82.
go back to reference Plant GT, Revesz T, Barnard RO, Harding AE, Gautier-Smith PC (1990) Familial cerebral amyloid angiopathy with nonneuritic amyloid plaque formation. Brain 113(Pt 3):721–747CrossRefPubMed Plant GT, Revesz T, Barnard RO, Harding AE, Gautier-Smith PC (1990) Familial cerebral amyloid angiopathy with nonneuritic amyloid plaque formation. Brain 113(Pt 3):721–747CrossRefPubMed
83.
84.
go back to reference Puri R, Suzuki T, Yamakawa K, Ganesh S (2009) Hyperphosphorylation and aggregation of Tau in laforin-deficient mice, an animal model for Lafora disease. J Biol Chem 284:22657–22663CrossRefPubMed Puri R, Suzuki T, Yamakawa K, Ganesh S (2009) Hyperphosphorylation and aggregation of Tau in laforin-deficient mice, an animal model for Lafora disease. J Biol Chem 284:22657–22663CrossRefPubMed
85.
go back to reference Revesz T, Holton JL, Doshi B, Anderton BH, Scaravilli F, Plant GT (1999) Cytoskeletal pathology in familial cerebral amyloid angiopathy (British type) with non-neuritic amyloid plaque formation. Acta Neuropathol 97:170–176CrossRefPubMed Revesz T, Holton JL, Doshi B, Anderton BH, Scaravilli F, Plant GT (1999) Cytoskeletal pathology in familial cerebral amyloid angiopathy (British type) with non-neuritic amyloid plaque formation. Acta Neuropathol 97:170–176CrossRefPubMed
86.
go back to reference 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 (2008) Amyloid-beta protein dimers isolated directly from Alzheimer’s brains impair synaptic plasticity and memory. Nat Med 14:837–842CrossRefPubMed 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 (2008) Amyloid-beta protein dimers isolated directly from Alzheimer’s brains impair synaptic plasticity and memory. Nat Med 14:837–842CrossRefPubMed
87.
go back to reference Sikorska B, Liberski PP, Sobow T, Budka H, Ironside JW (2009) Ultrastructural study of florid plaques in variant Creutzfeldt–Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt–Jakob disease and Gerstmann–Straussler–Scheinker disease. Neuropathol Appl Neurobiol 35:46–59CrossRefPubMed Sikorska B, Liberski PP, Sobow T, Budka H, Ironside JW (2009) Ultrastructural study of florid plaques in variant Creutzfeldt–Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt–Jakob disease and Gerstmann–Straussler–Scheinker disease. Neuropathol Appl Neurobiol 35:46–59CrossRefPubMed
88.
go back to reference Sobue K, Agarwal-Mawal A, Li W, Sun W, Miura Y, Paudel HK (2000) Interaction of neuronal Cdc2-like protein kinase with microtubule-associated protein tau. J Biol Chem 275:16673–16680CrossRefPubMed Sobue K, Agarwal-Mawal A, Li W, Sun W, Miura Y, Paudel HK (2000) Interaction of neuronal Cdc2-like protein kinase with microtubule-associated protein tau. J Biol Chem 275:16673–16680CrossRefPubMed
89.
go back to reference Tagliavini F, Prelli F, Ghiso J, Bugiani O, Serban D, Prusiner SB, Farlow MR, Ghetti B, Frangione B (1991) Amyloid protein of Gerstmann–Straussler–Scheinker disease (Indiana kindred) is an 11 kD fragment of prion protein with an N-terminal glycine at codon 58. EMBO J 10:513–519PubMed Tagliavini F, Prelli F, Ghiso J, Bugiani O, Serban D, Prusiner SB, Farlow MR, Ghetti B, Frangione B (1991) Amyloid protein of Gerstmann–Straussler–Scheinker disease (Indiana kindred) is an 11 kD fragment of prion protein with an N-terminal glycine at codon 58. EMBO J 10:513–519PubMed
90.
go back to reference Townsend M, Shankar GM, Mehta T, Walsh DM, Selkoe DJ (2006) Effects of secreted oligomers of amyloid beta-protein on hippocampal synaptic plasticity: a potent role for trimers. J Physiol 572:477–492CrossRefPubMed Townsend M, Shankar GM, Mehta T, Walsh DM, Selkoe DJ (2006) Effects of secreted oligomers of amyloid beta-protein on hippocampal synaptic plasticity: a potent role for trimers. J Physiol 572:477–492CrossRefPubMed
91.
go back to reference Tranchant C, Doh Ura K, Steinmetz G, Chevalier Y, Kitamoto T, Tateishi J, Warter JM (1991) Mutation of codon 117 of the prion gene in Gerstmann–Straussler–Scheinker disease. Rev Neurol Paris 147:274–278PubMed Tranchant C, Doh Ura K, Steinmetz G, Chevalier Y, Kitamoto T, Tateishi J, Warter JM (1991) Mutation of codon 117 of the prion gene in Gerstmann–Straussler–Scheinker disease. Rev Neurol Paris 147:274–278PubMed
92.
go back to reference Webb TE, Poulter M, Beck J, Uphill J, Adamson G, Campbell T, Linehan J, Powell C, Brandner S, Pal S, Siddique D, Wadsworth JD, Joiner S, Alner K, Petersen C, Hampson S, Rhymes C, Treacy C, Storey E, Geschwind MD, Nemeth AH, Wroe S, Collinge J, Mead S (2008) Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series. Brain 131:2632–2646CrossRefPubMed Webb TE, Poulter M, Beck J, Uphill J, Adamson G, Campbell T, Linehan J, Powell C, Brandner S, Pal S, Siddique D, Wadsworth JD, Joiner S, Alner K, Petersen C, Hampson S, Rhymes C, Treacy C, Storey E, Geschwind MD, Nemeth AH, Wroe S, Collinge J, Mead S (2008) Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series. Brain 131:2632–2646CrossRefPubMed
93.
go back to reference Williams DR, Holton JL, Strand C, Pittman A, de Silva R, Lees AJ, Revesz T (2007) Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain 130:1566–1576CrossRefPubMed Williams DR, Holton JL, Strand C, Pittman A, de Silva R, Lees AJ, Revesz T (2007) Pathological tau burden and distribution distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain 130:1566–1576CrossRefPubMed
94.
go back to reference Worster-Drought C, MW Hill TR (1933) Familial presenile dementia with spastic paralysis. J Neurol Psychopathol 14:27–34CrossRef Worster-Drought C, MW Hill TR (1933) Familial presenile dementia with spastic paralysis. J Neurol Psychopathol 14:27–34CrossRef
95.
go back to reference Yamada M, Itoh Y, Inaba A, Wada Y, Takashima M, Satoh S, Kamata T, Okeda R, Kayano T, Suematsu N, Kitamoto T, Otomo E, Matsushita M, Mizusawa H (1999) An inherited prion disease with a PrP P105L mutation: clinicopathologic and PrP heterogeneity (in process citation). Neurology 53:181–188PubMed Yamada M, Itoh Y, Inaba A, Wada Y, Takashima M, Satoh S, Kamata T, Okeda R, Kayano T, Suematsu N, Kitamoto T, Otomo E, Matsushita M, Mizusawa H (1999) An inherited prion disease with a PrP P105L mutation: clinicopathologic and PrP heterogeneity (in process citation). Neurology 53:181–188PubMed
96.
go back to reference Zhang M, Li J, Chakrabarty P, Bu B, Vincent I (2004) Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann–Pick type C mice. Am J Pathol 165:843–853PubMed Zhang M, Li J, Chakrabarty P, Bu B, Vincent I (2004) Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann–Pick type C mice. Am J Pathol 165:843–853PubMed
Metadata
Title
Tau, prions and Aβ: the triad of neurodegeneration
Authors
Lilla Reiniger
Ana Lukic
Jacqueline Linehan
Peter Rudge
John Collinge
Simon Mead
Sebastian Brandner
Publication date
01-01-2011
Publisher
Springer-Verlag
Published in
Acta Neuropathologica / Issue 1/2011
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI
https://doi.org/10.1007/s00401-010-0691-0

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