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Volumn 89, Issue , 2008, Pages 779-797

Biology and Neuropathology of Prion Diseases

Author keywords

[No Author keywords available]

Indexed keywords

ANIMAL; BRAIN; CLASSIFICATION; GENETICS; HUMAN; METABOLISM; MUTATION; PATHOLOGY; PATHOPHYSIOLOGY; PERIPHERAL NERVE; PRION; PRION DISEASE; REVIEW;

EID: 64449085391     PISSN: 00729752     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S0072-9752(07)01268-7     Document Type: Review
Times cited : (14)

References (115)
  • 1
    • 0842281643 scopus 로고    scopus 로고
    • Mammalian prion biology: one century of evolving concepts
    • Aguzzi A., and Polymenidou M. Mammalian prion biology: one century of evolving concepts. Cell 116 (2004) 313-327
    • (2004) Cell , vol.116 , pp. 313-327
    • Aguzzi, A.1    Polymenidou, M.2
  • 2
    • 0032905278 scopus 로고    scopus 로고
    • Fatal familial insomnia: a new Austrian family
    • Almer G., Hainfellner J.A., Brucke T., et al. Fatal familial insomnia: a new Austrian family. Brain 122 (1999) 5-16
    • (1999) Brain , vol.122 , pp. 5-16
    • Almer, G.1    Hainfellner, J.A.2    Brucke, T.3
  • 3
    • 0033562770 scopus 로고    scopus 로고
    • Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease
    • Alperovitch A., Zerr I., Pocchiari M., et al. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 353 (1999) 1673-1674
    • (1999) Lancet , vol.353 , pp. 1673-1674
    • Alperovitch, A.1    Zerr, I.2    Pocchiari, M.3
  • 4
    • 8044239656 scopus 로고    scopus 로고
    • Prion protein immunocytochemistry-UK five centre consensus report
    • Bell J.E., Gentleman S.M., Ironside J.W., et al. Prion protein immunocytochemistry-UK five centre consensus report. Neuropathol Appl Neurobiol 23 (1997) 26-35
    • (1997) Neuropathol Appl Neurobiol , vol.23 , pp. 26-35
    • Bell, J.E.1    Gentleman, S.M.2    Ironside, J.W.3
  • 5
    • 0028043661 scopus 로고
    • Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
    • Bessen R.A., and Marsh R.F. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol 68 (1994) 7859-7868
    • (1994) J Virol , vol.68 , pp. 7859-7868
    • Bessen, R.A.1    Marsh, R.F.2
  • 6
    • 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. Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol 5 (2006) 393-398
    • (2006) Lancet Neurol , vol.5 , pp. 393-398
    • Bishop, M.T.1    Hart, P.2    Aitchison, L.3
  • 7
    • 0038304770 scopus 로고    scopus 로고
    • Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK
    • Brandel J.P., Preece M., Brown P., et al. Distribution of codon 129 genotype in human growth hormone-treated CJD patients in France and the UK. Lancet 62 (2003) 128-130
    • (2003) Lancet , vol.62 , pp. 128-130
    • Brandel, J.P.1    Preece, M.2    Brown, P.3
  • 8
    • 0030054010 scopus 로고    scopus 로고
    • Normal host prion protein necessary for scrapie-induced neurotoxicity
    • Brandner S., Isenmann S., Raeber A., et al. Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 379 (1996) 339-343
    • (1996) Nature , vol.379 , pp. 339-343
    • Brandner, S.1    Isenmann, S.2    Raeber, A.3
  • 9
    • 0029825830 scopus 로고    scopus 로고
    • C) is required for scrapie spread within the central nervous system
    • C) is required for scrapie spread within the central nervous system. Proc Natl Acad Sci U S A 93 (1996) 13148-13151
    • (1996) Proc Natl Acad Sci U S A , vol.93 , pp. 13148-13151
    • Brandner, S.1    Raeber, A.2    Sailer, A.3
  • 10
    • 0035254585 scopus 로고    scopus 로고
    • Prion and prejudice: normal protein and the synapse
    • Brown D.R. Prion and prejudice: normal protein and the synapse. Trends Neurosci 24 (2001) 85-90
    • (2001) Trends Neurosci , vol.24 , pp. 85-90
    • Brown, D.R.1
  • 11
    • 0028235176 scopus 로고
    • Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease
    • Brown P., Gibbs Jr. C.J., Rodgers-Johnson P., et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 35 (1994) 513-529
    • (1994) Ann Neurol , vol.35 , pp. 513-529
    • Brown, P.1    Gibbs Jr., C.J.2    Rodgers-Johnson, P.3
  • 12
    • 33747040538 scopus 로고    scopus 로고
    • Iatrogenic Creutzfeldt-Jakob disease: the waning of an era
    • Brown P., Brandel J.P., Preece M., et al. Iatrogenic Creutzfeldt-Jakob disease: the waning of an era. Neurology 67 (2006) 389-393
    • (2006) Neurology , vol.67 , pp. 389-393
    • Brown, P.1    Brandel, J.P.2    Preece, M.3
  • 13
    • 0141515178 scopus 로고    scopus 로고
    • TSE strain variation
    • Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds), British Medical Bulletin, Oxford
    • Bruce M.E. TSE strain variation. In: Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds). Prions for physicians Vol. 66 (2003), British Medical Bulletin, Oxford 99-108
    • (2003) Prions for physicians , vol.66 , pp. 99-108
    • Bruce, M.E.1
  • 14
    • 0030775632 scopus 로고    scopus 로고
    • Transmissions to mice indicate that "new variant" CJD is caused by the BSE agent
    • Bruce E., Will R.G., Ironside J.W., et al. Transmissions to mice indicate that "new variant" CJD is caused by the BSE agent. Nature 389 (1997) 498-501
    • (1997) Nature , vol.389 , pp. 498-501
    • Bruce, E.1    Will, R.G.2    Ironside, J.W.3
  • 15
    • 0035928397 scopus 로고    scopus 로고
    • Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues
    • Bruce M.E., McConnell I., Will R.G., et al. Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues. Lancet 358 (2001) 208-209
    • (2001) Lancet , vol.358 , pp. 208-209
    • Bruce, M.E.1    McConnell, I.2    Will, R.G.3
  • 16
    • 66049109940 scopus 로고    scopus 로고
    • Neuropathology of prion disease
    • Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds), British Medical Bulletin, Oxford
    • Budka H. Neuropathology of prion disease. In: Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds). Prions for physicians Vol. 66 (2003), British Medical Bulletin, Oxford 213-239
    • (2003) Prions for physicians , vol.66 , pp. 213-239
    • Budka, H.1
  • 17
    • 0028876473 scopus 로고
    • Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies
    • Budka H., Aguzzi A., Brown P., et al. Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies. Brain Pathol 4 (1995) 459-466
    • (1995) Brain Pathol , vol.4 , pp. 459-466
    • Budka, H.1    Aguzzi, A.2    Brown, P.3
  • 18
    • 0027319326 scopus 로고
    • Mice devoid of PrP are resistant to scrapie
    • Bueler H., Aguzzi A., Sailer A., et al. Mice devoid of PrP are resistant to scrapie. Cell 73 (1993) 1339-1347
    • (1993) Cell , vol.73 , pp. 1339-1347
    • Bueler, H.1    Aguzzi, A.2    Sailer, A.3
  • 19
    • 0027531316 scopus 로고
    • Beta APP participates in PrP-amyloid plaques of Gerstmann-Straussler-Scheinker disease, Indiana kindred
    • Bugiani O., Giaccone G., Verga L., et al. Beta APP participates in PrP-amyloid plaques of Gerstmann-Straussler-Scheinker disease, Indiana kindred. J Neuropathol Exp Neurol 52 (1993) 64-70
    • (1993) J Neuropathol Exp Neurol , vol.52 , pp. 64-70
    • Bugiani, O.1    Giaccone, G.2    Verga, L.3
  • 20
    • 24744446203 scopus 로고    scopus 로고
    • Detection of prions in blood
    • Castilla J., Saa P., and Soto C. Detection of prions in blood. Nature Med 11 (2005) 982-985
    • (2005) Nature Med , vol.11 , pp. 982-985
    • Castilla, J.1    Saa, P.2    Soto, C.3
  • 21
    • 17444413067 scopus 로고    scopus 로고
    • In vitro generation of infectious scrapie prions
    • Castilla J., Saa P., Hetz C., et al. In vitro generation of infectious scrapie prions. Cell 121 (2005) 195-206
    • (2005) Cell , vol.121 , pp. 195-206
    • Castilla, J.1    Saa, P.2    Hetz, C.3
  • 22
    • 0141849856 scopus 로고    scopus 로고
    • Prion protein conversions: insight into mechanisms, TSE transmission barriers and strains
    • Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds), British Medical Bulletin, Oxford
    • Caughey B. Prion protein conversions: insight into mechanisms, TSE transmission barriers and strains. In: Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds). Prions for physicians Vol. 66 (2003), British Medical Bulletin, Oxford 109-120
    • (2003) Prions for physicians , vol.66 , pp. 109-120
    • Caughey, B.1
  • 23
    • 33750310849 scopus 로고    scopus 로고
    • Prions and their partners in crime
    • Caughey B., and Baron G.S. Prions and their partners in crime. Nature 443 (2006) 803-810
    • (2006) Nature , vol.443 , pp. 803-810
    • Caughey, B.1    Baron, G.S.2
  • 24
    • 0032573211 scopus 로고    scopus 로고
    • Phenotype-genotype studies in kuru: Implications for new variant Creutzfeldt-Jakob disease
    • Cervenakova L., Goldfarb L.G., Garruto R., et al. Phenotype-genotype studies in kuru: Implications for new variant Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A 95 (1998) 13239-13241
    • (1998) Proc Natl Acad Sci U S A , vol.95 , pp. 13239-13241
    • Cervenakova, L.1    Goldfarb, L.G.2    Garruto, R.3
  • 25
    • 0141520597 scopus 로고    scopus 로고
    • Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases
    • Collie D.A., Summers D.M., Sellar R.J., et al. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. Am J Neuroradiol 24 (2003) 1560-1569
    • (2003) Am J Neuroradiol , vol.24 , pp. 1560-1569
    • Collie, D.A.1    Summers, D.M.2    Sellar, R.J.3
  • 26
    • 0027997387 scopus 로고
    • Prion protein is necessary for normal synaptic function
    • Collinge J., Whittington M.A., Sidle K.C.L., et al. Prion protein is necessary for normal synaptic function. Nature 370 (1994) 295-297
    • (1994) Nature , vol.370 , pp. 295-297
    • Collinge, J.1    Whittington, M.A.2    Sidle, K.C.L.3
  • 27
    • 0029831213 scopus 로고    scopus 로고
    • Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
    • Collinge J., Sidle K.C.L., Meads J., et al. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 383 (1996) 685-690
    • (1996) Nature , vol.383 , pp. 685-690
    • Collinge, J.1    Sidle, K.C.L.2    Meads, J.3
  • 28
    • 33745440706 scopus 로고    scopus 로고
    • Kuru in the 21st century - an acquired human prion disease with very long incubation periods
    • Collinge J., Whitfield J., McKintosh E., et al. Kuru in the 21st century - an acquired human prion disease with very long incubation periods. Lancet 367 (2006) 2068-2074
    • (2006) Lancet , vol.367 , pp. 2068-2074
    • Collinge, J.1    Whitfield, J.2    McKintosh, E.3
  • 29
    • 0142184333 scopus 로고    scopus 로고
    • RNA molecules stimulate prion protein conversion
    • Deleault N.R., Lucassen R.W., and Supattapone S. RNA molecules stimulate prion protein conversion. Nature 425 (2003) 717-720
    • (2003) Nature , vol.425 , pp. 717-720
    • Deleault, N.R.1    Lucassen, R.W.2    Supattapone, S.3
  • 30
    • 0032565362 scopus 로고    scopus 로고
    • Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease
    • Deslys J., Jaegly A., Huillard d'Aignaux J., et al. Genotype at codon 129 and susceptibility to Creutzfeldt-Jakob disease. Lancet 351 (1998) 1251
    • (1998) Lancet , vol.351 , pp. 1251
    • Deslys, J.1    Jaegly, A.2    Huillard d'Aignaux, J.3
  • 31
    • 0016398968 scopus 로고
    • Possible person-to-person transmission of Creutzfeldt-Jakob disease
    • Duffy P., Wolf J., Collins G., et al. Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 290 (1974) 692-693
    • (1974) N Engl J Med , vol.290 , pp. 692-693
    • Duffy, P.1    Wolf, J.2    Collins, G.3
  • 32
    • 0027483615 scopus 로고
    • Neurotoxicity of a prion protein fragment
    • Forloni G., Angeretti N., Chiesa R., et al. Neurotoxicity of a prion protein fragment. Nature 362 (1993) 543-546
    • (1993) Nature , vol.362 , pp. 543-546
    • Forloni, G.1    Angeretti, N.2    Chiesa, R.3
  • 33
    • 78651041685 scopus 로고
    • Degenerative disease of the central nervous system in New Guinea. The endemic occurrence of "kuru" in the native population
    • Gajdusek D.C., and Zigas V. Degenerative disease of the central nervous system in New Guinea. The endemic occurrence of "kuru" in the native population. N Engl J Med 257 (1957) 974-978
    • (1957) N Engl J Med , vol.257 , pp. 974-978
    • Gajdusek, D.C.1    Zigas, V.2
  • 34
    • 0141514771 scopus 로고    scopus 로고
    • Sporadic and familial CJD: classification and characterisation
    • Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds), British Medical Bulletin, Oxford
    • Gambetti P., Kong Q., Zou W., et al. Sporadic and familial CJD: classification and characterisation. In: Weissman C., Aguzzi A., Dormont D., and Hunter N. (Eds). Prions for physicians Vol. 66 (2003), British Medical Bulletin, Oxford 213-239
    • (2003) Prions for physicians , vol.66 , pp. 213-239
    • Gambetti, P.1    Kong, Q.2    Zou, W.3
  • 35
    • 1642518575 scopus 로고    scopus 로고
    • Hereditary prion protein amyloidoses
    • Ghetti B., Tagliavini F., Takao M., et al. Hereditary prion protein amyloidoses. Clin Lab Med 23 (2003) 65-85
    • (2003) Clin Lab Med , vol.23 , pp. 65-85
    • Ghetti, B.1    Tagliavini, F.2    Takao, M.3
  • 36
    • 0035013510 scopus 로고    scopus 로고
    • Prion-induced neuronal damage-the mechanisms of neuronal destruction in the subacute spongiform encephalopathies
    • Giese A., and Kretzschmar H.A. Prion-induced neuronal damage-the mechanisms of neuronal destruction in the subacute spongiform encephalopathies. Curr Top Microbiol Immunol 253 (2001) 203-217
    • (2001) Curr Top Microbiol Immunol , vol.253 , pp. 203-217
    • Giese, A.1    Kretzschmar, H.A.2
  • 37
    • 0242361687 scopus 로고    scopus 로고
    • Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease
    • Glatzel M., Abela E., Maissen M., et al. Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. N Engl J Med 349 (2003) 1812-1820
    • (2003) N Engl J Med , vol.349 , pp. 1812-1820
    • Glatzel, M.1    Abela, E.2    Maissen, M.3
  • 38
  • 39
    • 33644493886 scopus 로고    scopus 로고
    • Structural studies of prion proteins
    • Crotty D., and Schaefer S. (Eds), Cold Spring Harbor Laboratory Press, New York
    • Govaerts C. Structural studies of prion proteins. In: Crotty D., and Schaefer S. (Eds). Prion Biology and Diseases. second edition (2004), Cold Spring Harbor Laboratory Press, New York 243-282
    • (2004) Prion Biology and Diseases. second edition , pp. 243-282
    • Govaerts, C.1
  • 40
    • 0030929562 scopus 로고    scopus 로고
    • Distribution of parvalbumin-immunoreactive neurons in brain correlates with hippocampal and temporal cortical pathology in Creutzfeldt-Jakob disease
    • Guentchev M., Hainfellner J.A., Trabattoni G.R., et al. Distribution of parvalbumin-immunoreactive neurons in brain correlates with hippocampal and temporal cortical pathology in Creutzfeldt-Jakob disease. J Neuropathol Exp Neurol 56 (1997) 1119-1124
    • (1997) J Neuropathol Exp Neurol , vol.56 , pp. 1119-1124
    • Guentchev, M.1    Hainfellner, J.A.2    Trabattoni, G.R.3
  • 41
    • 0031661397 scopus 로고    scopus 로고
    • Severe, early and selective loss of a subpopulation of gabaergic inhibitory neurons in experimental transmissible spongiform encephalopathies
    • Guentchev M., Groschup M.H., Kordek R., et al. Severe, early and selective loss of a subpopulation of gabaergic inhibitory neurons in experimental transmissible spongiform encephalopathies. Brain Pathol 8 (1998) 615-623
    • (1998) Brain Pathol , vol.8 , pp. 615-623
    • Guentchev, M.1    Groschup, M.H.2    Kordek, R.3
  • 42
    • 0033826082 scopus 로고    scopus 로고
    • Evidence for oxidative stress in experimental prion disease
    • Guentchev M., Voigtlander T., Haberler C., et al. Evidence for oxidative stress in experimental prion disease. Neurobiol Dis 7 (2000) 270-273
    • (2000) Neurobiol Dis , vol.7 , pp. 270-273
    • Guentchev, M.1    Voigtlander, T.2    Haberler, C.3
  • 43
    • 0028990981 scopus 로고
    • The original Gerstmann-Straussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype
    • Hainfellner J., Brantner-Inhaler S., Cervenakova L., et al. The original Gerstmann-Straussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype. Brain Pathol 5 (1995) 201-213
    • (1995) Brain Pathol , vol.5 , pp. 201-213
    • Hainfellner, J.1    Brantner-Inhaler, S.2    Cervenakova, L.3
  • 44
    • 0031053453 scopus 로고    scopus 로고
    • Pathology and immunocytochemistry of a kuru brain
    • Hainfellner J.A., Liberski P.P., Guiroy D.C., et al. Pathology and immunocytochemistry of a kuru brain. Brain Pathol 7 (1997) 547-553
    • (1997) Brain Pathol , vol.7 , pp. 547-553
    • Hainfellner, J.A.1    Liberski, P.P.2    Guiroy, D.C.3
  • 45
    • 3242660962 scopus 로고    scopus 로고
    • Cell biology of prions
    • Crotty D., and Schaefer S. (Eds), Cold Spring Harbor Laboratory Press, New York
    • Harris D.A. Cell biology of prions. In: Crotty D., and Schaefer S. (Eds). Prion Biology and Diseases. second edition (2004), Cold Spring Harbor Laboratory Press, New York 483-544
    • (2004) Prion Biology and Diseases. second edition , pp. 483-544
    • Harris, D.A.1
  • 46
    • 0034909904 scopus 로고    scopus 로고
    • Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype
    • Head M.W., Tissingh G., Uitdehaag B.M., et al. Sporadic Creutzfeldt-Jakob disease in a young Dutch valine homozygote: atypical molecular phenotype. Ann Neurol 50 (2001) 258-261
    • (2001) Ann Neurol , vol.50 , pp. 258-261
    • Head, M.W.1    Tissingh, G.2    Uitdehaag, B.M.3
  • 47
    • 2542618458 scopus 로고    scopus 로고
    • Prion protein geterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: U.K. Cases 1991-2002
    • Head M.W., Bunn T.J.R., Bishop M.T., et al. Prion protein geterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: U.K. Cases 1991-2002. Ann Neurol 55 (2004) 851-859
    • (2004) Ann Neurol , vol.55 , pp. 851-859
    • Head, M.W.1    Bunn, T.J.R.2    Bishop, M.T.3
  • 48
    • 1142267004 scopus 로고    scopus 로고
    • Peripheral tissue involvement in sporadic, iatrogenic and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative and biochemical study
    • Head M.W., Ritchie D., Smith N., et al. Peripheral tissue involvement in sporadic, iatrogenic and variant Creutzfeldt-Jakob disease: an immunohistochemical, quantitative and biochemical study. Am J Pathol 164 (2004) 143-153
    • (2004) Am J Pathol , vol.164 , pp. 143-153
    • Head, M.W.1    Ritchie, D.2    Smith, N.3
  • 49
    • 33749242953 scopus 로고    scopus 로고
    • Sporadic Creutzfeldt-Jakob disease: further twists and turns of a convoluted protein
    • Head M.W., and Ironside J.W. Sporadic Creutzfeldt-Jakob disease: further twists and turns of a convoluted protein. Brain 129 (2006) 2238-2240
    • (2006) Brain , vol.129 , pp. 2238-2240
    • Head, M.W.1    Ironside, J.W.2
  • 50
    • 33745311131 scopus 로고    scopus 로고
    • Dura mater-associated Creutzfeldt-Jakob disease - Experience from UK surveillance
    • Heath C.A., Barker R.A., Esmonde T.F.G., et al. Dura mater-associated Creutzfeldt-Jakob disease - Experience from UK surveillance. Neurol Neurosurg, Psych 77 (2006) 880-882
    • (2006) Neurol Neurosurg, Psych , vol.77 , pp. 880-882
    • Heath, C.A.1    Barker, R.A.2    Esmonde, T.F.G.3
  • 51
    • 0032488777 scopus 로고    scopus 로고
    • A transmembrane form of the prion protein in neurodegenerative disease
    • Hegde R.S., Mastrianni J.A., Scott M.R., et al. A transmembrane form of the prion protein in neurodegenerative disease. Science 279 (1998) 827-834
    • (1998) Science , vol.279 , pp. 827-834
    • Hegde, R.S.1    Mastrianni, J.A.2    Scott, M.R.3
  • 52
    • 0033570367 scopus 로고    scopus 로고
    • Evidence of presynaptic location and function of the prion protein
    • Herms J., Tings T., Gall S., et al. Evidence of presynaptic location and function of the prion protein. J Neurosci 19 (1999) 8866-8875
    • (1999) J Neurosci , vol.19 , pp. 8866-8875
    • Herms, J.1    Tings, T.2    Gall, S.3
  • 53
    • 33748369869 scopus 로고    scopus 로고
    • Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological review study
    • Hewitt P.E., Llewelyn C.A., Mackenzie J., et al. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological review study. Vox Sang 91 (2006) 221-230
    • (2006) Vox Sang , vol.91 , pp. 221-230
    • Hewitt, P.E.1    Llewelyn, C.A.2    Mackenzie, J.3
  • 54
    • 0033573778 scopus 로고    scopus 로고
    • Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples
    • Hill A.F., Butterworth R.J., Joiner S., et al. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 353 (1999) 183-189
    • (1999) Lancet , vol.353 , pp. 183-189
    • Hill, A.F.1    Butterworth, R.J.2    Joiner, S.3
  • 55
    • 0037677595 scopus 로고    scopus 로고
    • Molecular classification of sporadic Creutzfeldt-Jakob disease
    • Hill A.F., Joiner S., Wadsworth J.D.F., et al. Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 126 (2003) 1333-1346
    • (2003) Brain , vol.126 , pp. 1333-1346
    • Hill, A.F.1    Joiner, S.2    Wadsworth, J.D.F.3
  • 56
    • 0032578283 scopus 로고    scopus 로고
    • Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease
    • Hilton D.A., Fathers E., Edwards P., et al. Prion immunoreactivity in appendix before clinical onset of variant Creutzfeldt-Jakob disease. Lancet 352 (1998) 703-704
    • (1998) Lancet , vol.352 , pp. 703-704
    • Hilton, D.A.1    Fathers, E.2    Edwards, P.3
  • 57
    • 3242716876 scopus 로고    scopus 로고
    • Prevalence of lymphoreticular prion protein accumulation in UK tissue samples
    • Hilton D.A., Ghani A.C., Conyers L., et al. Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol 203 (2004) 733-739
    • (2004) J Pathol , vol.203 , pp. 733-739
    • Hilton, D.A.1    Ghani, A.C.2    Conyers, L.3
  • 58
    • 0024519771 scopus 로고
    • Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome
    • Hsiao K., Baker H.F., Crow T.J., et al. Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature 338 (1989) 342-345
    • (1989) Nature , vol.338 , pp. 342-345
    • Hsiao, K.1    Baker, H.F.2    Crow, T.J.3
  • 59
    • 0029910951 scopus 로고    scopus 로고
    • Creutzfeldt-Jakob disease
    • Ironside J.W. Creutzfeldt-Jakob disease. Brain Pathol 6 (1996) 379-388
    • (1996) Brain Pathol , vol.6 , pp. 379-388
    • Ironside, J.W.1
  • 60
    • 0033930868 scopus 로고    scopus 로고
    • Laboratory diagnosis of variant Creutzfeldt-Jakob disease
    • Ironside J.W., Head M.W., Bell J.E., et al. Laboratory diagnosis of variant Creutzfeldt-Jakob disease. Histopathology 37 (2000) 1-9
    • (2000) Histopathology , vol.37 , pp. 1-9
    • Ironside, J.W.1    Head, M.W.2    Bell, J.E.3
  • 61
    • 0036219975 scopus 로고    scopus 로고
    • Pathological diagnosis of variant Creutzfeldt-Jakob disease
    • Ironside J.W., McCardle L., Horsburgh A., et al. Pathological diagnosis of variant Creutzfeldt-Jakob disease. APMIS 11 (2002) 79-87
    • (2002) APMIS , vol.11 , pp. 79-87
    • Ironside, J.W.1    McCardle, L.2    Horsburgh, A.3
  • 63
    • 33646898809 scopus 로고    scopus 로고
    • Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study
    • Ironside J.W., Bishop M.T., Connolly K., et al. Variant Creutzfeldt-Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 332 (2006) 1186-1188
    • (2006) BMJ , vol.332 , pp. 1186-1188
    • Ironside, J.W.1    Bishop, M.T.2    Connolly, K.3
  • 64
    • 0033583190 scopus 로고    scopus 로고
    • Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations
    • Jackson G.S., Hosszu L.L.P., Power A., et al. Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. Science 283 (1999) 1935-1937
    • (1999) Science , vol.283 , pp. 1935-1937
    • Jackson, G.S.1    Hosszu, L.L.P.2    Power, A.3
  • 65
    • 0037385558 scopus 로고    scopus 로고
    • Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene
    • King A., Doey L., Rossor M., et al. Phenotypic variability in the brains of a family with a prion disease characterized by a 144-base pair insertion in the prion protein gene. Neuropathol Appl Neurobiol 29 (2003) 98-105
    • (2003) Neuropathol Appl Neurobiol , vol.29 , pp. 98-105
    • King, A.1    Doey, L.2    Rossor, M.3
  • 66
    • 0027236933 scopus 로고
    • An amber mutation of prion protein in Gerstmann-Straussler syndrome with mutant prp plaques
    • Kitamoto T., Iizuka R., and Tateishi J. An amber mutation of prion protein in Gerstmann-Straussler syndrome with mutant prp plaques. Biochem Biophys Res Commun 192 (1993) 525-531
    • (1993) Biochem Biophys Res Commun , vol.192 , pp. 525-531
    • Kitamoto, T.1    Iizuka, R.2    Tateishi, J.3
  • 67
    • 0027956109 scopus 로고
    • Cell-free formation of protease-resistant prion protein
    • Kocisko D.A., Come J.H., Priola S.A., et al. Cell-free formation of protease-resistant prion protein. Nature 370 (1994) 471-474
    • (1994) Nature , vol.370 , pp. 471-474
    • Kocisko, D.A.1    Come, J.H.2    Priola, S.A.3
  • 68
    • 0036901006 scopus 로고    scopus 로고
    • Disease-associated prion protein in vessel walls
    • Koperek O., Kovacs G.G., Ritchie D., et al. Disease-associated prion protein in vessel walls. Am J Pathol 161 (2002) 1979-1984
    • (2002) Am J Pathol , vol.161 , pp. 1979-1984
    • Koperek, O.1    Kovacs, G.G.2    Ritchie, D.3
  • 69
    • 0036132894 scopus 로고    scopus 로고
    • Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes
    • Kovacs G.G., Head M.W., Hegyi I., et al. Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes. Brain Pathol 12 (2002) 1-11
    • (2002) Brain Pathol , vol.12 , pp. 1-11
    • Kovacs, G.G.1    Head, M.W.2    Hegyi, I.3
  • 70
    • 0036459944 scopus 로고    scopus 로고
    • Mutations of the prion protein gene phenotypic spectrum
    • Kovacs G.G., Trabattoni G., Hainfellner J.A., et al. Mutations of the prion protein gene phenotypic spectrum. J Neurol 249 (2002) 1567-1582
    • (2002) J Neurol , vol.249 , pp. 1567-1582
    • Kovacs, G.G.1    Trabattoni, G.2    Hainfellner, J.A.3
  • 71
    • 1642456624 scopus 로고    scopus 로고
    • Complement activation in human prion disease
    • Kovacs G.G., Gasque P., Strobel T., et al. Complement activation in human prion disease. Neurobiol Dis 15 (2004) 21-28
    • (2004) Neurobiol Dis , vol.15 , pp. 21-28
    • Kovacs, G.G.1    Gasque, P.2    Strobel, T.3
  • 72
    • 11144231706 scopus 로고    scopus 로고
    • Subcellular localization of disease-associated prion protein in the human brain
    • Kovacs G.G., Preusser M., Strohschneider M., et al. Subcellular localization of disease-associated prion protein in the human brain. Am J Pathol 166 (2005) 287-294
    • (2005) Am J Pathol , vol.166 , pp. 287-294
    • Kovacs, G.G.1    Preusser, M.2    Strohschneider, M.3
  • 73
    • 20944445878 scopus 로고    scopus 로고
    • Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia and Canada
    • Ladogana A., Puopolo M., Croes E.A., et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia and Canada. Neurology 64 (2005) 1586-1591
    • (2005) Neurology , vol.64 , pp. 1586-1591
    • Ladogana, A.1    Puopolo, M.2    Croes, E.A.3
  • 74
    • 0342505312 scopus 로고    scopus 로고
    • Ancestral origins and worldwide distribution of the PRNP 200K mutation causing familial Creutzfeldt-Jakob disease
    • Lee H.S., Sambuughin N., Cervenakova L., et al. Ancestral origins and worldwide distribution of the PRNP 200K mutation causing familial Creutzfeldt-Jakob disease. Am J Hum Genet 64 (1999) 1063-1070
    • (1999) Am J Hum Genet , vol.64 , pp. 1063-1070
    • Lee, H.S.1    Sambuughin, N.2    Cervenakova, L.3
  • 75
    • 3442889359 scopus 로고    scopus 로고
    • Synthetic mammalian prions
    • Legname G., Baskakov I.V., Nguyen H., et al. Synthetic mammalian prions. Science 305 (2004) 673-676
    • (2004) Science , vol.305 , pp. 673-676
    • Legname, G.1    Baskakov, I.V.2    Nguyen, H.3
  • 76
    • 0026720748 scopus 로고
    • Comparative ultrastructural neuropathology of naturally occurring bovine spongiform encephalopathy and experimentally induced scrapie and Creutzfeldt-Jakob Disease
    • Liberski P.P., Yanagihara R., Wells G.A., et al. Comparative ultrastructural neuropathology of naturally occurring bovine spongiform encephalopathy and experimentally induced scrapie and Creutzfeldt-Jakob Disease. J Comp Pathol 106 (1992) 361-381
    • (1992) J Comp Pathol , vol.106 , pp. 361-381
    • Liberski, P.P.1    Yanagihara, R.2    Wells, G.A.3
  • 77
    • 1142273431 scopus 로고    scopus 로고
    • Possible transmission of variant Creutzfeldt-Jakob disase by blood transfusion
    • Llewelyn C.A., Hewitt P.E., Knight R.S., et al. Possible transmission of variant Creutzfeldt-Jakob disase by blood transfusion. Lancet 363 (2004) 417-421
    • (2004) Lancet , vol.363 , pp. 417-421
    • Llewelyn, C.A.1    Hewitt, P.E.2    Knight, R.S.3
  • 78
    • 0031804370 scopus 로고    scopus 로고
    • Comparative neuropathology of kuru with the new variant of Creutzfeldt-Jakob disease: evidence for strain of agent predominating over genotype of host
    • McLean C.A., Ironside J.W., Alpers M.P., et al. Comparative neuropathology of kuru with the new variant of Creutzfeldt-Jakob disease: evidence for strain of agent predominating over genotype of host. Brain Pathol 8 (1998) 428-437
    • (1998) Brain Pathol , vol.8 , pp. 428-437
    • McLean, C.A.1    Ironside, J.W.2    Alpers, M.P.3
  • 79
    • 3042594982 scopus 로고    scopus 로고
    • Prion protein accumulation and neuroprotection in hypoxic brain damage
    • McLennan N.F., Brennan P.M., McNeill A., et al. Prion protein accumulation and neuroprotection in hypoxic brain damage. Am J Pathol 165 (2004) 227-235
    • (2004) Am J Pathol , vol.165 , pp. 227-235
    • McLennan, N.F.1    Brennan, P.M.2    McNeill, A.3
  • 80
    • 0037195647 scopus 로고    scopus 로고
    • Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol
    • Ma J., Wollmann R., and Lindquist S. Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol. Science 298 (2002) 1781-1785
    • (2002) Science , vol.298 , pp. 1781-1785
    • Ma, J.1    Wollmann, R.2    Lindquist, S.3
  • 81
    • 0017871609 scopus 로고
    • Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change
    • Masters C.L., and Richardson E.P. Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease). The nature and progression of spongiform change. Brain 101 (1978) 333-344
    • (1978) Brain , vol.101 , pp. 333-344
    • Masters, C.L.1    Richardson, E.P.2
  • 82
    • 0019803861 scopus 로고
    • Neuropathology of Creutzfeldt-Jakob disease in Japan. With special reference to the panencephalopathic type
    • Mizutani T. Neuropathology of Creutzfeldt-Jakob disease in Japan. With special reference to the panencephalopathic type. Acta Pathol Jpn 31 (1981) 903-922
    • (1981) Acta Pathol Jpn , vol.31 , pp. 903-922
    • Mizutani, T.1
  • 84
    • 0034665847 scopus 로고    scopus 로고
    • Signal transduction through prion protein
    • Mouillet-Richard S., Ermonval M., Chebassier C., et al. Signal transduction through prion protein. Science 289 (2000) 1925-1928
    • (2000) Science , vol.289 , pp. 1925-1928
    • Mouillet-Richard, S.1    Ermonval, M.2    Chebassier, C.3
  • 85
    • 0026703146 scopus 로고
    • Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease
    • Neufeld M.Y., Josiphov J., and Korczyn A.D. Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease. Muscle Nerve 15 (1992) 1234-1239
    • (1992) Muscle Nerve , vol.15 , pp. 1234-1239
    • Neufeld, M.Y.1    Josiphov, J.2    Korczyn, A.D.3
  • 86
    • 0000612171 scopus 로고
    • Subacute spongiform encephalopathy - a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy)
    • Nevin S., McMenemey W.H., Behrman S., et al. Subacute spongiform encephalopathy - a subacute form of encephalopathy attributable to vascular dysfunction (spongiform cerebral atrophy). Brain 83 (1960) 519-563
    • (1960) Brain , vol.83 , pp. 519-563
    • Nevin, S.1    McMenemey, W.H.2    Behrman, S.3
  • 87
  • 88
    • 0032493453 scopus 로고    scopus 로고
    • Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Straussler-Scheinker disease
    • Parchi P., Chen S.G., Brown P., et al. Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Straussler-Scheinker disease. Proc Natl Acad Sci U S A 95 (1998) 8322-8327
    • (1998) Proc Natl Acad Sci U S A , vol.95 , pp. 8322-8327
    • Parchi, P.1    Chen, S.G.2    Brown, P.3
  • 89
    • 7144253121 scopus 로고    scopus 로고
    • Molecular pathology of fatal familial insomnia
    • Parchi P., Petersen R.B., Chen S.G., et al. Molecular pathology of fatal familial insomnia. Brain Pathology 8 (1998) 539-548
    • (1998) Brain Pathology , vol.8 , pp. 539-548
    • Parchi, P.1    Petersen, R.B.2    Chen, S.G.3
  • 90
    • 0032816292 scopus 로고    scopus 로고
    • Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects
    • Parchi P., Giese A., Capellari S., et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46 (1999) 224-233
    • (1999) Ann Neurol , vol.46 , pp. 224-233
    • Parchi, P.1    Giese, A.2    Capellari, S.3
  • 91
    • 12944253111 scopus 로고    scopus 로고
    • Genetic influence on the structural variations of the abnormal prion protein
    • Parchi P., Zou W., Wang W., et al. Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A 97 (2000) 10168-10172
    • (2000) Proc Natl Acad Sci U S A , vol.97 , pp. 10168-10172
    • Parchi, P.1    Zou, W.2    Wang, W.3
  • 92
    • 4043157677 scopus 로고    scopus 로고
    • Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient
    • Peden A.H., Head M.W., Ritchie D.L., et al. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364 (2004) 477-479
    • (2004) Lancet , vol.364 , pp. 477-479
    • Peden, A.H.1    Head, M.W.2    Ritchie, D.L.3
  • 93
    • 33644651130 scopus 로고    scopus 로고
    • Sc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease
    • Sc in the skeletal muscle of patients with variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease. Am J Pathol 168 (2006) 927-935
    • (2006) Am J Pathol , vol.168 , pp. 927-935
    • Peden, A.H.1    Ritchie, D.L.2    Head, M.W.3
  • 94
    • 0031754291 scopus 로고    scopus 로고
    • Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity
    • Piccardo P., Dloughy S.R., Lievens P.M.J., et al. Phenotypic variability of Gerstmann-Straussler-Scheinker disease is associated with prion protein heterogeneity. J Neuropathol Exp Neurol 57 (1998) 979-988
    • (1998) J Neuropathol Exp Neurol , vol.57 , pp. 979-988
    • Piccardo, P.1    Dloughy, S.R.2    Lievens, P.M.J.3
  • 95
    • 0031789527 scopus 로고    scopus 로고
    • Rapid acquisition of ß-sheet structure in the prion protein prior to multimer formation
    • Post K., Pitschke M., Schafer O., et al. Rapid acquisition of ß-sheet structure in the prion protein prior to multimer formation. Biol Chem 379 (1998) 1307-1317
    • (1998) Biol Chem , vol.379 , pp. 1307-1317
    • Post, K.1    Pitschke, M.2    Schafer, O.3
  • 96
    • 0002181235 scopus 로고    scopus 로고
    • An introduction to prion biology and diseases
    • Crotty D., and Schaefer S. (Eds), Cold Spring Harbor Laboratory Press, New York
    • Prusiner S.B. An introduction to prion biology and diseases. In: Crotty D., and Schaefer S. (Eds). Prion Biology and Diseases. second edition (2004), Cold Spring Harbor Laboratory Press, New York 1-87
    • (2004) Prion Biology and Diseases. second edition , pp. 1-87
    • Prusiner, S.B.1
  • 97
    • 3442879322 scopus 로고    scopus 로고
    • Development of the prion concept
    • Crotty D., and Schaefer S. (Eds), Cold Spring Harbor Laboratory Press, New York
    • Prusiner S.B. Development of the prion concept. In: Crotty D., and Schaefer S. (Eds). Prion Biology and Diseases. second edition (2004), Cold Spring Harbor Laboratory Press, New York 89-141
    • (2004) Prion Biology and Diseases. second edition , pp. 89-141
    • Prusiner, S.B.1
  • 98
    • 0033853890 scopus 로고    scopus 로고
    • Polymorphism at codon 129 of PRNP affects the phenotypic expression of Creutzfeldt-Jakob disease linked to E200K mutation
    • Puoti G., Rossi G., Giaccone G., et al. Polymorphism at codon 129 of PRNP affects the phenotypic expression of Creutzfeldt-Jakob disease linked to E200K mutation. Ann Neurol 48 (2000) 269-270
    • (2000) Ann Neurol , vol.48 , pp. 269-270
    • Puoti, G.1    Rossi, G.2    Giaccone, G.3
  • 99
    • 0031436335 scopus 로고    scopus 로고
    • The human 37-kda laminin receptor precursor interacts with the prion protein in eukaryotic cells
    • Rieger R., Edenhofer F., Lasmezas C.I., et al. The human 37-kda laminin receptor precursor interacts with the prion protein in eukaryotic cells. Nature Med 3 (1997) 1383-1388
    • (1997) Nature Med , vol.3 , pp. 1383-1388
    • Rieger, R.1    Edenhofer, F.2    Lasmezas, C.I.3
  • 100
    • 4344684277 scopus 로고    scopus 로고
    • Advances in the detection of prion protein in peripheral tissues of variant Creutzfeldt-Jakob disease patients using paraffin-embedded tissue blotting
    • Ritchie D.L., Head M.W., and Ironside J.W. Advances in the detection of prion protein in peripheral tissues of variant Creutzfeldt-Jakob disease patients using paraffin-embedded tissue blotting. Neuropathol Appl Neurobiol 30 (2004) 360-368
    • (2004) Neuropathol Appl Neurobiol , vol.30 , pp. 360-368
    • Ritchie, D.L.1    Head, M.W.2    Ironside, J.W.3
  • 101
    • 0035859102 scopus 로고    scopus 로고
    • Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding
    • Saborio G.P., Permanne B., and Soto C. Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 411 (2001) 810-813
    • (2001) Nature , vol.411 , pp. 810-813
    • Saborio, G.P.1    Permanne, B.2    Soto, C.3
  • 102
    • 18544376071 scopus 로고    scopus 로고
    • Prion protein recruits its neuronal receptor NCAM to lipid rafts to activate p59fyn and to enhance neurite outgrowth
    • Santuccione A., Sytnyk V., Leshchyns'ka I., et al. Prion protein recruits its neuronal receptor NCAM to lipid rafts to activate p59fyn and to enhance neurite outgrowth. J Cell Biol 169 (2005) 341-354
    • (2005) J Cell Biol , vol.169 , pp. 341-354
    • Santuccione, A.1    Sytnyk, V.2    Leshchyns'ka, I.3
  • 103
    • 0141841804 scopus 로고    scopus 로고
    • Association of an 11-12 kda protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases
    • Satoh K., Muramoto T., Tanaka T., et al. Association of an 11-12 kda protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases. J Gen Virol 84 (2003) 2885-2893
    • (2003) J Gen Virol , vol.84 , pp. 2885-2893
    • Satoh, K.1    Muramoto, T.2    Tanaka, T.3
  • 104
    • 17044382824 scopus 로고    scopus 로고
    • Transgenetic investigations of the species barrier and prion strains
    • Crotty D., and Schaefer S. (Eds), Cold Spring Harbor Laboratory Press, New York
    • Scott M. Transgenetic investigations of the species barrier and prion strains. In: Crotty D., and Schaefer S. (Eds). Prion Biology and Diseases. second edition (2004), Cold Spring Harbor Laboratory Press, New York 435-482
    • (2004) Prion Biology and Diseases. second edition , pp. 435-482
    • Scott, M.1
  • 105
    • 0032989733 scopus 로고    scopus 로고
    • Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting
    • Shimizu S., Hoshi T., Homma M., et al. Creutzfeldt-Jakob disease with florid-type plaques after cadaveric dura mater grafting. Arch Neurol 56 (1999) 357-363
    • (1999) Arch Neurol , vol.56 , pp. 357-363
    • Shimizu, S.1    Hoshi, T.2    Homma, M.3
  • 106
    • 0348077417 scopus 로고    scopus 로고
    • A neuronal isoform of the Aplysia CFEB has prion-like properties
    • Si K., Lindquist S., and Kandel E.R. A neuronal isoform of the Aplysia CFEB has prion-like properties. Cell 115 (2003) 879-891
    • (2003) Cell , vol.115 , pp. 879-891
    • Si, K.1    Lindquist, S.2    Kandel, E.R.3
  • 107
    • 0141765736 scopus 로고    scopus 로고
    • Creutzfeldt-Jakob disease in United Kingdom patients treated with human pituitary growth hormone
    • Swerdlow A.J., Higgins C.D., Adlard P., et al. Creutzfeldt-Jakob disease in United Kingdom patients treated with human pituitary growth hormone. Neurology 61 (2003) 783-791
    • (2003) Neurology , vol.61 , pp. 783-791
    • Swerdlow, A.J.1    Higgins, C.D.2    Adlard, P.3
  • 108
    • 0034932717 scopus 로고    scopus 로고
    • Studies on peptide fragments of prion proteins
    • Tagliavini F., Forloni G., D'Ursi P., et al. Studies on peptide fragments of prion proteins. Adv Protein Chem 57 (2001) 171-201
    • (2001) Adv Protein Chem , vol.57 , pp. 171-201
    • Tagliavini, F.1    Forloni, G.2    D'Ursi, P.3
  • 109
    • 12644272790 scopus 로고    scopus 로고
    • Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity
    • Telling G.C., Parchi P., DeArmond S.J., et al. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science 274 (1996) 2079-2082
    • (1996) Science , vol.274 , pp. 2079-2082
    • Telling, G.C.1    Parchi, P.2    DeArmond, S.J.3
  • 110
    • 1642387431 scopus 로고    scopus 로고
    • Coexistence of CJD and Alzheimer's disease: an autopsy case showing typical clinical features of CJD
    • Tsuchiya K., Yagishita S., Ikeda K., et al. Coexistence of CJD and Alzheimer's disease: an autopsy case showing typical clinical features of CJD. Neuropathology 24 (2004) 46-55
    • (2004) Neuropathology , vol.24 , pp. 46-55
    • Tsuchiya, K.1    Yagishita, S.2    Ikeda, K.3
  • 111
    • 15444352327 scopus 로고    scopus 로고
    • Prion encephalopathy with insertion of octapeptide repeats: the number of repeats determines the type of cerebellar deposits
    • Vital C., Gray F., Vital A., et al. Prion encephalopathy with insertion of octapeptide repeats: the number of repeats determines the type of cerebellar deposits. Neuropathol Appl Neurobiol 24 (1998) 125-130
    • (1998) Neuropathol Appl Neurobiol , vol.24 , pp. 125-130
    • Vital, C.1    Gray, F.2    Vital, A.3
  • 112
    • 0037183530 scopus 로고    scopus 로고
    • Sporadic Creutzfeldt-Jakob disease and surgery: a case-control study using community controls
    • Ward H.J., Everington D., Croes E.A., et al. Sporadic Creutzfeldt-Jakob disease and surgery: a case-control study using community controls. Neurology 59 (2002) 543-548
    • (2002) Neurology , vol.59 , pp. 543-548
    • Ward, H.J.1    Everington, D.2    Croes, E.A.3
  • 113
  • 114
    • 0342951746 scopus 로고    scopus 로고
    • A new variant Creutzfeldt-Jakob disease in the UK
    • Will R.G., Ironside J.W., Zeidler M., et al. A new variant Creutzfeldt-Jakob disease in the UK. Lancet 347 (1996) 921-925
    • (1996) Lancet , vol.347 , pp. 921-925
    • Will, R.G.1    Ironside, J.W.2    Zeidler, M.3
  • 115
    • 0035145614 scopus 로고    scopus 로고
    • Increased levels of oxidative stress markers detected in the brains of mice devoid of prion protein
    • Wong B., Liu T., Ruliang L., et al. Increased levels of oxidative stress markers detected in the brains of mice devoid of prion protein. J Neurochem 76 (2001) 565-572
    • (2001) J Neurochem , vol.76 , pp. 565-572
    • Wong, B.1    Liu, T.2    Ruliang, L.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.