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Volumn 119, Issue 2, 2011, Pages 251-261

Biochemical and strain properties of CJD prions: Complexity versus simplicity

Author keywords

Creutzfeldt Jakob; prion; PrP; strain; transmissible spongiform encephalopathy

Indexed keywords

PRION PROTEIN;

EID: 80053566142     PISSN: 00223042     EISSN: 14714159     Source Type: Journal    
DOI: 10.1111/j.1471-4159.2011.07399.x     Document Type: Review
Times cited : (21)

References (116)
  • 1
    • 67649286622 scopus 로고    scopus 로고
    • Cell biology: Beyond the prion principle
    • Aguzzi A., (2009) Cell biology: beyond the prion principle. Nature 459, 924-925.
    • (2009) Nature , vol.459 , pp. 924-925
    • Aguzzi, A.1
  • 3
    • 38949108662 scopus 로고    scopus 로고
    • Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD
    • Beringue V., Le Dur A., Tixador P., et al. (2008) Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD. PLoS ONE 3, e1419.
    • (2008) PLoS ONE , vol.3
    • Beringue, V.1    Le Dur, A.2    Tixador, P.3
  • 4
    • 0028997297 scopus 로고
    • Non-genetic propagation of strain-specific properties of scrapie prion protein
    • Bessen R. A., Kocisko D. A., Raymond G. J., Nandan S., Lansbury P. T., and, Caughey B., (1995) Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 375, 698-700.
    • (1995) Nature , vol.375 , pp. 698-700
    • Bessen, R.A.1    Kocisko, D.A.2    Raymond, G.J.3    Nandan, S.4    Lansbury, P.T.5    Caughey, B.6
  • 5
    • 33646059507 scopus 로고    scopus 로고
    • Predicting susceptibility and incubation time of human-to-human transmission of vCJD
    • Bishop M. T., Hart P., Aitchison L., et al. (2006) Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol. 5, 393-398.
    • (2006) Lancet Neurol. , vol.5 , pp. 393-398
    • Bishop, M.T.1    Hart, P.2    Aitchison, L.3
  • 6
    • 77955344991 scopus 로고    scopus 로고
    • Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties
    • Bishop M. T., Will R. G., and, Manson J. C., (2010) Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc. Natl. Acad. Sci. USA 107, 12005-12010.
    • (2010) Proc. Natl. Acad. Sci. USA , vol.107 , pp. 12005-12010
    • Bishop, M.T.1    Will, R.G.2    Manson, J.C.3
  • 7
    • 0038304770 scopus 로고    scopus 로고
    • Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK
    • DOI 10.1016/S0140-6736(03)13867-6
    • Brandel J. P., Preece M., Brown P., Croes E., Laplanche J. L., Agid Y., Will R., and, Alperovitch A., (2003) Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK. Lancet 362, 128-130. (Pubitemid 36831026)
    • (2003) Lancet , vol.362 , Issue.9378 , pp. 128-130
    • Brandel, J.-P.1    Preece, M.2    Brown, P.3    Croes, E.4    Laplanche, J.-L.5    Agid, Y.6    Will, R.7    Alperovitch, A.8
  • 8
    • 65249149613 scopus 로고    scopus 로고
    • Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain
    • Brandel J. P., Heath C. A., Head M. W., et al. (2009) Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain. Ann. Neurol. 65, 249-256.
    • (2009) Ann. Neurol. , vol.65 , pp. 249-256
    • Brandel, J.P.1    Heath, C.A.2    Head, M.W.3
  • 9
    • 77749251805 scopus 로고    scopus 로고
    • Variant or sporadic Creutzfeldt-Jakob disease?
    • author reply 889-890.
    • Brandel J. P., Galanaud D., Freeman L., Laplanche J. L., and, Haik S., (2010) Variant or sporadic Creutzfeldt-Jakob disease? Lancet 375, 889; author reply 889-890.
    • (2010) Lancet , vol.375 , pp. 889
    • Brandel, J.P.1    Galanaud, D.2    Freeman, L.3    Laplanche, J.L.4    Haik, S.5
  • 10
    • 78651243687 scopus 로고    scopus 로고
    • Diversity of prion diseases: (No) strains attached?
    • Brandner S., (2011) Diversity of prion diseases: (no) strains attached? Acta Neuropathol. 121, 1-4.
    • (2011) Acta Neuropathol. , vol.121 , pp. 1-4
    • Brandner, S.1
  • 12
    • 33747040538 scopus 로고    scopus 로고
    • Iatrogenic Creutzfeldt-Jakob disease: The waning of an era
    • DOI 10.1212/01.wnl.0000231528.65069.3f, PII 0000611420060808000009
    • Brown P., Brandel J. P., Preece M., and, Sato T., (2006) Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology 67, 389-393. (Pubitemid 44214958)
    • (2006) Neurology , vol.67 , Issue.3 , pp. 389-393
    • Brown, P.1    Brandel, J.-P.2    Preese, M.3    Sato, T.4
  • 13
    • 0017059781 scopus 로고
    • Cerebral amyloidosis in scrapie in the mouse: effect of agent strain and mouse genotype
    • Bruce M. E., Dickinson A. G., and, Fraser H., (1976) Cerebral amyloidosis in scrapie in the mouse: effect of agent strain and mouse genotype. Neuropathol. Appl. Neurobiol. 2, 471-478. (Pubitemid 8011708)
    • (1976) Neuropathol. Appl. Neurobiol. , vol.2 , Issue.6 , pp. 471-478
    • Bruce, M.E.1    Dickinson, A.G.2    Fraser, H.3
  • 15
    • 0035928397 scopus 로고    scopus 로고
    • Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues
    • DOI 10.1016/S0140-6736(01)05411-3
    • Bruce M. E., McConnell I., Will R. G., and, Ironside J. W., (2001) Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues. Lancet 358, 208-209. (Pubitemid 32718547)
    • (2001) Lancet , vol.358 , Issue.9277 , pp. 208-209
    • Bruce, M.E.1    McConnell, I.2    Will, R.G.3    Ironside, J.W.4
  • 17
    • 70349937836 scopus 로고    scopus 로고
    • Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: Its effect on the phenotype and prion-type characteristics
    • Cali I., Castellani R., Alshekhlee A., et al. (2009) Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics. Brain 132, 2643-2658.
    • (2009) Brain , vol.132 , pp. 2643-2658
    • Cali, I.1    Castellani, R.2    Alshekhlee, A.3
  • 19
    • 17444413067 scopus 로고    scopus 로고
    • In vitro generation of infectious scrapie prions
    • DOI 10.1016/j.cell.2005.02.011
    • Castilla J., Saa P., Hetz C., and, Soto C., (2005) In vitro generation of infectious scrapie prions. Cell 121, 195-206. (Pubitemid 40546388)
    • (2005) Cell , vol.121 , Issue.2 , pp. 195-206
    • Castilla, J.1    Saa, P.2    Hetz, C.3    Soto, C.4
  • 20
    • 50249157526 scopus 로고    scopus 로고
    • Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions
    • Castilla J., Gonzalez-Romero D., Saa P., Morales R., De Castro J., and, Soto C., (2008a) Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions. Cell 134, 757-768.
    • (2008) Cell , vol.134 , pp. 757-768
    • Castilla, J.1    Gonzalez-Romero, D.2    Saa, P.3    Morales, R.4    De Castro, J.5    Soto, C.6
  • 22
    • 0033610870 scopus 로고    scopus 로고
    • Strain-dependent differences in β-sheet conformations of abnormal prion protein
    • DOI 10.1074/jbc.273.48.32230
    • Caughey B., Raymond G. J., and, Bessen R. A., (1998) Strain-dependent differences in beta-sheet conformations of abnormal prion protein. J. Biol. Chem. 27, 32230-32235. (Pubitemid 29177169)
    • (1998) Journal of Biological Chemistry , vol.273 , Issue.48 , pp. 32230-32235
    • Caughey, B.1    Raymond, G.J.2    Bessen, R.A.3
  • 23
    • 67650077008 scopus 로고    scopus 로고
    • Transmission and spreading of tauopathy in transgenic mouse brain
    • Clavaguera F., Bolmont T., Crowther R. A., et al. (2009) Transmission and spreading of tauopathy in transgenic mouse brain. Nat. Cell Biol. 11, 909-913.
    • (2009) Nat. Cell Biol. , vol.11 , pp. 909-913
    • Clavaguera, F.1    Bolmont, T.2    Crowther, R.A.3
  • 24
    • 0033600407 scopus 로고    scopus 로고
    • Variant Creutzfeldt-Jakob disease
    • DOI 10.1016/S0140-6736(99)05128-4
    • Collinge J., (1999) Variant Creutzfeldt-Jakob disease. Lancet 354, 317-323. (Pubitemid 29348751)
    • (1999) Lancet , vol.354 , Issue.9175 , pp. 317-323
    • Collinge, J.1
  • 25
    • 77952969480 scopus 로고    scopus 로고
    • Medicine prion strain mutation and selection
    • Collinge J., (2010) Medicine prion strain mutation and selection. Science 328, 1111-1112.
    • (2010) Science , vol.328 , pp. 1111-1112
    • Collinge, J.1
  • 26
    • 36049020231 scopus 로고    scopus 로고
    • A general model of prion strains and their pathogenicity
    • DOI 10.1126/science.1138718
    • Collinge J., and, Clarke A. R., (2007) A general model of prion strains and their pathogenicity. Science 318, 930-936. (Pubitemid 350098981)
    • (2007) Science , vol.318 , Issue.5852 , pp. 930-936
    • Collinge, J.1    Clarke, A.R.2
  • 27
    • 0029831213 scopus 로고    scopus 로고
    • Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
    • DOI 10.1038/383685a0
    • Collinge J., Sidle K. C. L., Meads J., Ironside J., and, Hill A. F., (1996) Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 383, 685-690. (Pubitemid 26360638)
    • (1996) Nature , vol.383 , Issue.6602 , pp. 685-690
    • Collinge, J.1    Sidle, K.C.L.2    Meads, J.3    Ironside, J.4    Hill, A.F.5
  • 28
    • 33745440706 scopus 로고    scopus 로고
    • Kuru in the 21st century-an acquired human prion disease with very long incubation periods
    • DOI 10.1016/S0140-6736(06)68930-7, PII S0140673606689307
    • Collinge J., Whitfield J., McKintosh E., Beck J., Mead S., Thomas D. J., and, Alpers M. P., (2006) Kuru in the 21st century-an acquired human prion disease with very long incubation periods. Lancet 367, 2068-2074. (Pubitemid 43949290)
    • (2006) Lancet , vol.367 , Issue.9528 , pp. 2068-2074
    • Collinge, J.1    Whitfield, J.2    McKintosh, E.3    Beck, J.4    Mead, S.5    Thomas, D.J.6    Alpers, M.P.7
  • 29
    • 51549111981 scopus 로고    scopus 로고
    • Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate
    • Comoy E. E., Casalone C., Lescoutra-Etchegaray N., et al. (2008) Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate. PLoS ONE 3, e3017.
    • (2008) PLoS ONE , vol.3
    • Comoy, E.E.1    Casalone, C.2    Lescoutra-Etchegaray, N.3
  • 30
    • 37049039483 scopus 로고    scopus 로고
    • Prion strain- and species-dependent effects of antiprion molecules in primary neuronal cultures
    • DOI 10.1128/JVI.01502-07
    • Cronier S., Beringue V., Bellon A., Peyrin J. M., and, Laude H., (2007) Prion strain- and species-dependent effects of antiprion molecules in primary neuronal cultures. J. Virol. 81, 13794-13800. (Pubitemid 350247872)
    • (2007) Journal of Virology , vol.81 , Issue.24 , pp. 13794-13800
    • Cronier, S.1    Beringue, V.2    Bellon, A.3    Peyrin, J.-M.4    Laude, H.5
  • 31
    • 33750576527 scopus 로고    scopus 로고
    • Prions: Protein only or something more? Overview of potential prion cofactors
    • DOI 10.1385/JMN:29:3:195, PII JMN293195
    • Fasano C., Campana V., and, Zurzolo C., (2006) Prions: protein only or something more? Overview of potential prion cofactors J. Mol. Neurosci. 29, 195-214. (Pubitemid 44684495)
    • (2006) Journal of Molecular Neuroscience , vol.29 , Issue.3 , pp. 195-214
    • Fasano, C.1    Campana, V.2    Zurzolo, C.3
  • 32
    • 69449087379 scopus 로고    scopus 로고
    • Loss of cerebellar granule neurons is associated with punctate but not with large focal deposits of prion protein in Creutzfeldt-Jakob disease
    • Faucheux B. A., Privat N., Brandel J. P., Sazdovitch V., Laplanche J. L., Maurage C. A., Hauw J. J., and, Haik S., (2009) Loss of cerebellar granule neurons is associated with punctate but not with large focal deposits of prion protein in Creutzfeldt-Jakob disease. J. Neuropathol. Exp. Neurol. 68, 892-901.
    • (2009) J. Neuropathol. Exp. Neurol. , vol.68 , pp. 892-901
    • Faucheux, B.A.1    Privat, N.2    Brandel, J.P.3    Sazdovitch, V.4    Laplanche, J.L.5    Maurage, C.A.6    Hauw, J.J.7    Haik, S.8
  • 33
    • 79960231821 scopus 로고    scopus 로고
    • Quantification of surviving cerebellar granule neurones and abnormal prion protein (PrP(Sc)) deposition in sporadic Creutzfeldt-Jakob disease supports a pathogenic role for small PrP(Sc) deposits common to the various molecular subtypes
    • Faucheux B. A., Morain E., Diouron V., et al. (2011) Quantification of surviving cerebellar granule neurones and abnormal prion protein (PrP(Sc)) deposition in sporadic Creutzfeldt-Jakob disease supports a pathogenic role for small PrP(Sc) deposits common to the various molecular subtypes. Neuropathol. Appl. Neurobiol. 37, 500-512.
    • (2011) Neuropathol. Appl. Neurobiol. , vol.37 , pp. 500-512
    • Faucheux, B.A.1    Morain, E.2    Diouron, V.3
  • 34
    • 63649160214 scopus 로고    scopus 로고
    • Conformational diversity of wild-type Tau fibrils specified by templated conformation change
    • Frost B., Ollesch J., Wille H., and, Diamond M. I., (2009) Conformational diversity of wild-type Tau fibrils specified by templated conformation change. J. Biol. Chem. 284, 3546-3551.
    • (2009) J. Biol. Chem. , vol.284 , pp. 3546-3551
    • Frost, B.1    Ollesch, J.2    Wille, H.3    Diamond, M.I.4
  • 36
    • 78651240044 scopus 로고    scopus 로고
    • Molecular biology and pathology of prion strains in sporadic human prion diseases
    • Gambetti P., Cali I., Notari S., Kong Q., Zou W. Q., and, Surewicz W. K., (2011) Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol. 121, 79-90.
    • (2011) Acta Neuropathol. , vol.121 , pp. 79-90
    • Gambetti, P.1    Cali, I.2    Notari, S.3    Kong, Q.4    Zou, W.Q.5    Surewicz, W.K.6
  • 41
    • 7444254877 scopus 로고    scopus 로고
    • Brain targeting through the autonomous nervous system: Lessons from prion diseases
    • Haik S., Faucheux B. A., and, Hauw J. J., (2004b) Brain targeting through the autonomous nervous system: lessons from prion diseases. Trends Mol. Med. 10, 107-112.
    • (2004) Trends Mol. Med. , vol.10 , pp. 107-112
    • Haik, S.1    Faucheux, B.A.2    Hauw, J.J.3
  • 46
    • 67649995088 scopus 로고    scopus 로고
    • Pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant Creutzfeldt-Jakob disease transmission
    • Head M. W., Yull H. M., Ritchie D. L., Bishop M. T., and, Ironside J. W., (2009b) Pathological investigation of the first blood donor and recipient pair linked by transfusion-associated variant Creutzfeldt-Jakob disease transmission. Neuropathol. Appl. Neurobiol. 35, 433-436.
    • (2009) Neuropathol. Appl. Neurobiol. , vol.35 , pp. 433-436
    • Head, M.W.1    Yull, H.M.2    Ritchie, D.L.3    Bishop, M.T.4    Ironside, J.W.5
  • 48
    • 0031015906 scopus 로고    scopus 로고
    • Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy
    • DOI 10.1016/S0140-6736(97)24002-X
    • Hill A. F., Zeidler M., Ironside J., and, Collinge J., (1997b) Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet 349, 99-100. (Pubitemid 27023429)
    • (1997) Lancet , vol.349 , Issue.9045 , pp. 99-100
    • Hill, A.F.1    Zeidler, M.2    Ironside, J.3    Collinge, J.4
  • 51
    • 33646898809 scopus 로고    scopus 로고
    • Variant Creutzfeldt-Jakob disease: Prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study
    • DOI 10.1136/bmj.38804.511644.55
    • Ironside J. W., Bishop M. T., Connolly K., Hegazy D., Lowrie S., Le Grice M., Ritchie D. L., McCardle L. M., and, Hilton D. A., (2006) Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 332, 1186-1188. (Pubitemid 44365449)
    • (2006) British Medical Journal , vol.332 , Issue.7551 , pp. 1186-1188
    • Ironside, J.W.1    Bishop, M.T.2    Connolly, K.3    Hegazy, D.4    Lowrie, S.5    Le Grice, M.6    Ritchie, D.L.7    McCardle, L.M.8    Hilton, D.A.9
  • 52
    • 77955295345 scopus 로고    scopus 로고
    • The first case of protease-sensitive prionopathy (PSPr) in the Netherlands: A patient with an unusual GSS-like clinical phenotype
    • Jansen C., Head M. W., van Gool W. A., Baas F., Yull H., Ironside J. W., and, Rozemuller A. J., (2010) The first case of protease-sensitive prionopathy (PSPr) in the Netherlands: a patient with an unusual GSS-like clinical phenotype. J. Neurol. Neurosurg. Psychiatry 81, 1052-1055.
    • (2010) J. Neurol. Neurosurg. Psychiatry , vol.81 , pp. 1052-1055
    • Jansen, C.1    Head, M.W.2    Van Gool, W.A.3    Baas, F.4    Yull, H.5    Ironside, J.W.6    Rozemuller, A.J.7
  • 58
    • 33746129139 scopus 로고    scopus 로고
    • Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum
    • DOI 10.1111/j.1440-1789.2006.00683.x
    • Kawauchi Y., Kamitani T., Yagishita S., Kitamoto T., and, Kishida H., (2006) Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum. Neuropathology 26, 313-317. (Pubitemid 44086834)
    • (2006) Neuropathology , vol.26 , Issue.4 , pp. 313-317
    • Kawauchi, Y.1    Kamitani, T.2    Yagishita, S.3    Kitamoto, T.4    Kishida, H.5
  • 59
    • 35649016744 scopus 로고    scopus 로고
    • Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain
    • DOI 10.1074/jbc.M704597200
    • Kobayashi A., Asano M., Mohri S., and, Kitamoto T., (2007) Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain. J. Biol. Chem. 282, 30022-30028. (Pubitemid 350035250)
    • (2007) Journal of Biological Chemistry , vol.282 , Issue.41 , pp. 30022-30028
    • Kobayashi, A.1    Asano, M.2    Mohri, S.3    Kitamoto, T.4
  • 60
    • 54149085116 scopus 로고    scopus 로고
    • Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients
    • Kobayashi A., Arima K., Ogawa M., Murata M., Fukuda T., and, Kitamoto T., (2008) Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients. Acta Neuropathol. 116, 561-566.
    • (2008) Acta Neuropathol. , vol.116 , pp. 561-566
    • Kobayashi, A.1    Arima, K.2    Ogawa, M.3    Murata, M.4    Fukuda, T.5    Kitamoto, T.6
  • 61
    • 77949407881 scopus 로고    scopus 로고
    • Experimental verification of a traceback phenomenon in prion infection
    • Kobayashi A., Sakuma N., Matsuura Y., Mohri S., Aguzzi A., and, Kitamoto T., (2010) Experimental verification of a traceback phenomenon in prion infection. J. Virol. 84, 3230-3238.
    • (2010) J. Virol. , vol.84 , pp. 3230-3238
    • Kobayashi, A.1    Sakuma, N.2    Matsuura, Y.3    Mohri, S.4    Aguzzi, A.5    Kitamoto, T.6
  • 70
    • 75649120399 scopus 로고    scopus 로고
    • Conformational diversity in prion protein variants influences intermolecular beta-sheet formation
    • Lee S., Antony L., Hartmann R., Knaus K. J., Surewicz K., Surewicz W. K., and, Yee V. C., (2010) Conformational diversity in prion protein variants influences intermolecular beta-sheet formation. EMBO J. 29, 251-262.
    • (2010) EMBO J. , vol.29 , pp. 251-262
    • Lee, S.1    Antony, L.2    Hartmann, R.3    Knaus, K.J.4    Surewicz, K.5    Surewicz, W.K.6    Yee, V.C.7
  • 71
    • 50949100224 scopus 로고    scopus 로고
    • Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease - Implications for the dissemination and the diagnosis of human prion strains
    • Levavasseur E., Laffont-Proust I., Morain E., et al. (2008) Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease - implications for the dissemination and the diagnosis of human prion strains. PLoS ONE 3, e2786.
    • (2008) PLoS ONE , vol.3
    • Levavasseur, E.1    Laffont-Proust, I.2    Morain, E.3
  • 73
    • 1142273431 scopus 로고    scopus 로고
    • Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion
    • DOI 10.1016/S0140-6736(04)15486-X
    • Llewelyn C. A., Hewitt P. E., Knight R. S., Amar K., Cousens S., Mackenzie J., and, Will R. G., (2004) Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 363, 417-421. (Pubitemid 38210057)
    • (2004) Lancet , vol.363 , Issue.9407 , pp. 417-421
    • Llewelyn, C.A.1    Hewitt, P.E.2    Knight, R.S.G.3    Amar, K.4    Cousens, S.5    MacKenzie, J.6    Will, R.G.7
  • 74
    • 33745897112 scopus 로고    scopus 로고
    • Long-duration sCJD with PRNP codon 129 methionine homozygosity and cerebral cortical plaques
    • DOI 10.1212/01.wnl.0000217913.37108.94, PII 0000611420060627000038
    • Lo R. Y., Shyu W. C., and, Li H., (2006) Long-duration sCJD with PRNP codon 129 methionine homozygosity and cerebral cortical plaques. Neurology 66, 1944-1945. (Pubitemid 44049837)
    • (2006) Neurology , vol.66 , Issue.12 , pp. 1944-1945
    • Lo, R.Y.-Y.1    Shyu, W.C.2    Li, H.3
  • 75
    • 0242363656 scopus 로고    scopus 로고
    • Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis
    • DOI 10.1126/science.1090187
    • Mallucci G., Dickinson A., Linehan J., Klohn P. C., Brandner S., and, Collinge J., (2003) Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science 302, 871-874. (Pubitemid 37339628)
    • (2003) Science , vol.302 , Issue.5646 , pp. 871-874
    • Mallucci, G.1    Dickinson, A.2    Linehan, J.3    Klohn, P.-C.4    Brandner, S.5    Collinge, J.6
  • 78
    • 34249993165 scopus 로고    scopus 로고
    • The prion strain phenomenon: Molecular basis and unprecedented features
    • DOI 10.1016/j.bbadis.2006.12.006, PII S0925443906002729, Prion-Related Disorders
    • Morales R., Abid K., and, Soto C., (2007) The prion strain phenomenon: molecular basis and unprecedented features. Biochim. Biophys. Acta 1772, 681-691. (Pubitemid 46887653)
    • (2007) Biochimica et Biophysica Acta - Molecular Basis of Disease , vol.1772 , Issue.6 , pp. 681-691
    • Morales, R.1    Abid, K.2    Soto, C.3
  • 79
    • 0028902465 scopus 로고
    • Developmental expression of the prion protein gene in glial cells
    • Moser M., Colello R. J., Pott U., and, Oesch B., (1995) Developmental expression of the prion protein gene in glial cells. Neuron 14, 509-517.
    • (1995) Neuron , vol.14 , pp. 509-517
    • Moser, M.1    Colello, R.J.2    Pott, U.3    Oesch, B.4
  • 80
    • 33645778583 scopus 로고    scopus 로고
    • Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles
    • Nonno R., Bari M. A., Cardone F., et al. (2006) Efficient transmission and characterization of creutzfeldt-jakob disease strains in bank voles. PLoS Pathog. 2, e12.
    • (2006) PLoS Pathog. , vol.2
    • Nonno, R.1    Bari, M.A.2    Cardone, F.3
  • 81
    • 1942533390 scopus 로고    scopus 로고
    • Sc core generated by protease digestion: Implications for strain typing and molecular classification of CJD
    • DOI 10.1074/jbc.M313220200
    • Notari S., Capellari S., Giese A., Westner I., Baruzzi A., Ghetti B., Gambetti P., Kretzschmar H. A., and, Parchi P., (2004) Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J. Biol. Chem. 279, 16797-16804. (Pubitemid 38509382)
    • (2004) Journal of Biological Chemistry , vol.279 , Issue.16 , pp. 16797-16804
    • Notari, S.1    Capellari, S.2    Giese, A.3    Westner, I.4    Baruzzi, A.5    Ghetti, B.6    Gambetti, P.7    Kretzschmar, H.A.8    Parchi, P.9
  • 82
    • 57649233091 scopus 로고    scopus 로고
    • Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease
    • Notari S., Strammiello R., Capellari S., et al. (2008) Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J. Biol. Chem. 283, 30557-30565.
    • (2008) J. Biol. Chem. , vol.283 , pp. 30557-30565
    • Notari, S.1    Strammiello, R.2    Capellari, S.3
  • 83
    • 69149088365 scopus 로고    scopus 로고
    • Is Parkinson's disease a prion disorder?
    • Olanow C. W., and, Prusiner S. B., (2009) Is Parkinson's disease a prion disorder? Proc. Natl. Acad. Sci. USA 106, 12571-12572.
    • (2009) Proc. Natl. Acad. Sci. USA , vol.106 , pp. 12571-12572
    • Olanow, C.W.1    Prusiner, S.B.2
  • 85
    • 0030953939 scopus 로고    scopus 로고
    • Typing prion isoforms
    • Parchi P., Capellari S., Chen S. G., et al. (1997) Typing prion isoforms. Nature 386, 232-233.
    • (1997) Nature , vol.386 , pp. 232-233
    • Parchi, P.1    Capellari, S.2    Chen, S.G.3
  • 87
    • 12944253111 scopus 로고    scopus 로고
    • Genetic influence on the structural variations of the abnormal prion protein
    • Parchi P., Zou W., Wang W., et al. (2000) Genetic influence on the structural variations of the abnormal prion protein. Proc. Natl. Acad. Sci. USA 97, 10168-10172.
    • (2000) Proc. Natl. Acad. Sci. USA , vol.97 , pp. 10168-10172
    • Parchi, P.1    Zou, W.2    Wang, W.3
  • 88
    • 66949127502 scopus 로고    scopus 로고
    • Inter-laboratory assessment of PrP(Sc) typing in Creutzfeldt-Jakob disease: A western blot study within the NeuroPrion Consortium
    • Parchi P., Notari S., Weber P., et al. (2009a) Inter-laboratory assessment of PrP(Sc) typing in Creutzfeldt-Jakob disease: a western blot study within the NeuroPrion Consortium. Brain Pathol. 9, 384-391.
    • (2009) Brain Pathol. , vol.9 , pp. 384-391
    • Parchi, P.1    Notari, S.2    Weber, P.3
  • 89
    • 70449524296 scopus 로고    scopus 로고
    • Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: An updated classification
    • Parchi P., Strammiello R., Notari S., et al. (2009b) Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol. 118, 659-671.
    • (2009) Acta Neuropathol. , vol.118 , pp. 659-671
    • Parchi, P.1    Strammiello, R.2    Notari, S.3
  • 90
    • 77957660770 scopus 로고    scopus 로고
    • Agent strain variation in human prion disease: Insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease
    • Parchi P., Cescatti M., Notari S., et al. (2010) Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease. Brain 133, 3030-3042.
    • (2010) Brain , vol.133 , pp. 3030-3042
    • Parchi, P.1    Cescatti, M.2    Notari, S.3
  • 91
    • 79959442590 scopus 로고    scopus 로고
    • Phenotypic variability of sporadic human prion disease and its molecular basis: Past, present, and future
    • Parchi P., Strammiello R., Giese A., and, Kretzschmar H., (2011) Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future. Acta Neuropathol. 121, 91-112.
    • (2011) Acta Neuropathol. , vol.121 , pp. 91-112
    • Parchi, P.1    Strammiello, R.2    Giese, A.3    Kretzschmar, H.4
  • 92
    • 4043157677 scopus 로고    scopus 로고
    • Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient
    • DOI 10.1016/S0140-6736(04)16811-6, PII S0140673604168116
    • Peden A. H., Head M. W., Ritchie D. L., Bell J. E., and, Ironside J. W., (2004) Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364, 527-529. (Pubitemid 39070413)
    • (2004) Lancet , vol.364 , Issue.9433 , pp. 527-529
    • Peden, A.H.1    Head, M.W.2    Ritchie, D.L.3    Bell, P.J.E.4    Ironside, P.J.W.5
  • 94
    • 0032763817 scopus 로고    scopus 로고
    • Sporadic Creutzfeldt-Jakob disease: Co-occurrence of different types of PrP(Sc) in the same brain
    • Puoti G., Giaccone G., Rossi G., Canciani B., Bugiani O., and, Tagliavini F., (1999) Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain. Neurology 53, 2173-2176.
    • (1999) Neurology , vol.53 , pp. 2173-2176
    • Puoti, G.1    Giaccone, G.2    Rossi, G.3    Canciani, B.4    Bugiani, O.5    Tagliavini, F.6
  • 95
    • 33745879463 scopus 로고    scopus 로고
    • Presymptomatic detection of prions in blood
    • DOI 10.1126/science.1129051
    • Saa P., Castilla J., and, Soto C., (2006) Presymptomatic detection of prions in blood. Science 313, 92-94. (Pubitemid 44051260)
    • (2006) Science , vol.313 , Issue.5783 , pp. 92-94
    • Saa, P.1    Castilla, J.2    Soto, C.3
  • 96
    • 0035859102 scopus 로고    scopus 로고
    • Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding
    • DOI 10.1038/35081095
    • Saborio G. P., Permanne B., and, Soto C., (2001) Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 411, 810-813. (Pubitemid 32588105)
    • (2001) Nature , vol.411 , Issue.6839 , pp. 810-813
    • Saborio, G.P.1    Permanne, B.2    Soto, C.3
  • 100
    • 70349858126 scopus 로고    scopus 로고
    • Ultrastructures and strain comparison of under-glycosylated scrapie prion fibrils
    • Sim V. L., and, Caughey B., (2009) Ultrastructures and strain comparison of under-glycosylated scrapie prion fibrils. Neurobiol. Aging 30, 2031-2042.
    • (2009) Neurobiol. Aging , vol.30 , pp. 2031-2042
    • Sim, V.L.1    Caughey, B.2
  • 101
    • 0344844524 scopus 로고    scopus 로고
    • Humanized Knock-in Mice Expressing Chimeric Prion Protein Showed Varied Susceptibility to Different Human Prions
    • Taguchi Y., Mohri S., Ironside J. W., Muramoto T., and, Kitamoto T., (2003) Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am. J. Pathol. 163, 2585-2593. (Pubitemid 37466499)
    • (2003) American Journal of Pathology , vol.163 , Issue.6 , pp. 2585-2593
    • Taguchi, Y.1    Mohri, S.2    Ironside, J.W.3    Muramoto, T.4    Kitamoto, T.5
  • 102
    • 80053572163 scopus 로고
    • Transgenic mice expressing human and chimeric human-mouse prion proteins carrying the codon 102 mutation of GSS
    • (A. a. o. Neurology ed.) Neurology, Seattle, Washington
    • Telling G. C., Foster D., Yang S. L., DeArmond S. J., and, Prusiner S. B., (1995) Transgenic mice expressing human and chimeric human-mouse prion proteins carrying the codon 102 mutation of GSS, in 47ème meeting annuel de l'académie américaine de Neurologie (A. a. o. Neurology ed.), Vol. 45, pp. 521. P. Neurology, Seattle, Washington.
    • (1995) 47ème Meeting Annuel de l'Académie Américaine de Neurologie , vol.45 , pp. 521
    • Telling, G.C.1    Foster, D.2    Yang, S.L.3    Dearmond, S.J.4    Prusiner, S.B.5
  • 104
    • 4043137988 scopus 로고    scopus 로고
    • Discriminating scrapie and bovine spongiform encephalopathy isolates by infrared spectroscopy of pathological prion protein
    • DOI 10.1074/jbc.M403730200
    • Thomzig A., Spassov S., Friedrich M., Naumann D., and, Beekes M., (2004) Discriminating scrapie and bovine spongiform encephalopathy isolates by infrared spectroscopy of pathological prion protein. J. Biol. Chem. 279, 33847-33854. (Pubitemid 39063034)
    • (2004) Journal of Biological Chemistry , vol.279 , Issue.32 , pp. 33847-33854
    • Thomzig, A.1    Spassov, S.2    Friedrich, M.3    Naumann, D.4    Beekes, M.5
  • 106
    • 42949128703 scopus 로고    scopus 로고
    • Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt-Jakob disease
    • Uro-Coste E., Cassard H., Simon S., et al. (2008) Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt-Jakob disease. PLoS Pathog. 4, e1000029.
    • (2008) PLoS Pathog. , vol.4
    • Uro-Coste, E.1    Cassard, H.2    Simon, S.3
  • 109
    • 9444267651 scopus 로고    scopus 로고
    • The state of the prion
    • DOI 10.1038/nrmicro1025
    • Weissmann C., (2004) The state of the prion. Nat. Rev. Microbiol. 2, 861-871. (Pubitemid 39561804)
    • (2004) Nature Reviews Microbiology , vol.2 , Issue.11 , pp. 861-871
    • Weissmann, C.1
  • 116
    • 77955302607 scopus 로고    scopus 로고
    • Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein
    • Zou W. Q., Puoti G., Xiao X., et al. (2010) Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann. Neurol. 68, 162-172.
    • (2010) Ann. Neurol. , vol.68 , pp. 162-172
    • Zou, W.Q.1    Puoti, G.2    Xiao, X.3


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