Prusiner SB, Scott MR, DeArmond SJ, Cohen FE: Prion protein biology. Cell 1998, 93: 337–348.
Article
CAS
PubMed
Google Scholar
Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG, et al.: Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996, 39: 767–778.
Article
CAS
PubMed
Google Scholar
Parchi P, Capellari S, Chen SG, Petersen RB, Gambetti P, Kopp N, et al.: Typing prion isoforms. Nature 1997, 386: 232–234.
Article
CAS
PubMed
Google Scholar
Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, et al.: Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999, 46: 224–233.
Article
CAS
PubMed
Google Scholar
Parchi P, Strammiello R, Giese A, Kretzschmar H: Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future. Acta Neuropathol 2011, 121: 91–112.
Article
CAS
PubMed
Google Scholar
Parchi P, Zou W, Wang W, Brown P, Capellari S, Ghetti B, et al.: Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci USA 2000, 97: 10168–10172.
Article
PubMed Central
CAS
PubMed
Google Scholar
Collinge J, Sidle KC, Meads J, Ironside J, Hill AF: Molecular analysis of prion strain variation and the aetiology of ‘new variant’ CJD. Nature 1996, 383: 685–690.
Article
CAS
PubMed
Google Scholar
Hill AF, Joiner S, Wadsworth JD, Sidle KC, Bell JE, Budka H, et al.: Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 2003, 126: 1333–1346.
Article
PubMed
Google Scholar
Parchi P, Strammiello R, Notari S, Giese A, Langeveld JP, Ladogana A, et al.: Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol 2009, 118: 659–671.
Article
PubMed Central
CAS
PubMed
Google Scholar
Parchi P, de Boni L, Saverioni D, Cohen ML, Ferrer I, Gambetti P, et al.: Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol 2012, 124: 517–529.
Article
PubMed Central
PubMed
Google Scholar
Notari S, Capellari S, Giese A, Westner I, Baruzzi A, Ghetti B, et al.: Effects of different experimental conditions on the PrPSc core generated by protease digestion. J Biol Chem 2004, 279: 16797–16804.
Article
CAS
PubMed
Google Scholar
Notari S, Capellari S, Langeveld J, Giese A, Strammiello R, Gambetti P, et al.: A refined method for molecular typing reveals that co-occurrence of PrPSc types in Creutzfeldt-Jakob disease is not the rule. Lab Invest 2007, 87: 1103–1112.
Article
CAS
PubMed
Google Scholar
Bishop MT, Will RG, Manson JC: Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 2010, 107: 12005–12010.
Article
PubMed Central
CAS
PubMed
Google Scholar
Kitamoto T, Shin R-W, Doh-ura K, Tomokane N, Miyazono M, Muramoto T, et al.: Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease. Am J Pathol 1992, 140: 1285–1294.
PubMed Central
CAS
PubMed
Google Scholar
Taguchi Y, Mohri S, Ironside JW, Muramoto T, Kitamoto T: Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol 2003, 163: 2585–2593.
Article
PubMed Central
CAS
PubMed
Google Scholar
Kitamoto T, Ohta M, Doh-ura K, Hitoshi S, Terao Y, Tateishi J: Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome. Biochem Biophys Res Commun 1993, 191: 709–714.
Article
CAS
PubMed
Google Scholar
Kitamoto T, Mohri S, Ironside JW, Miyoshi I, Tanaka T, Kitamoto N, et al.: Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions. Biochem Biophys Res Commun 2002, 294: 280–286.
Article
CAS
PubMed
Google Scholar
Asano M, Mohri S, Ironside JW, Ito M, Tamaoki N, Kitamoto T: vCJD prion acquires altered virulence through trans-species infection. Biochem Biophys Res Commun 2006, 342: 293–299.
Article
CAS
PubMed
Google Scholar
Kascsak RJ, Rubenstein R, Merz PA, Tonna-DeMasi M, Fersko R, Carp RI, et al.: Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins. J Virol 1987, 61: 3688–3693.
PubMed Central
CAS
PubMed
Google Scholar
Muramoto T, Tanaka T, Kitamoto N, Sano C, Hayashi Y, Kutomi T, et al.: Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu. Neurosci Lett 2000, 288: 179–182.
Article
CAS
PubMed
Google Scholar
Satoh K, Muramoto T, Tanaka T, Kitamoto N, Ironside JW, Nagashima K, 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 2003, 84: 2885–2893.
Article
CAS
PubMed
Google Scholar
Kitamoto T, Muramoto T, Hilbich C, Beyreuther K, Tateishi J: N-terminal sequence of prion protein is also integrated into kuru plaques in patients with Gerstmann-Sträussler syndrome. Brain Res 1991, 545: 319–321.
Article
CAS
PubMed
Google Scholar
Grathwohl KUD, Horiuchi M, Ishiguro N, Shinagawa M: Improvement of PrPSc
-detection in mouse spleen early at the preclinical stage of scrapie with collagenase-completed tissue homogenization and Sarkosyl-NaCl extraction of PrPSc
. Arch Virol 1996, 141: 1863–1874.
Article
CAS
PubMed
Google Scholar
Ikeda S, Kobayashi A, Kitamoto T: Thr but Asn of the N-glycosylation sites of PrP is indispensable for its misfolding. Biochem Biophys Res Commun 2008, 369: 1195–1198.
Article
CAS
PubMed
Google Scholar
Kobayashi A, Sakuma N, Matsuura Y, Mohri S, Aguzzi A, Kitamoto T: Experimental verification of a traceback phenomenon in prion infection. J Virol 2010, 84: 3230–3238.
Article
PubMed Central
CAS
PubMed
Google Scholar
Polymenidou M, Stoeck K, Glatzel M, Vey M, Bellon A, Aguzzi A: Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol 2005, 4: 805–814.
Article
CAS
PubMed
Google Scholar
Yull HM, Ritchie DL, Langeveld JP, van Zijderveld FG, Bruce ME, Ironside JW, et al.: Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol 2006, 168: 151–157.
Article
PubMed Central
CAS
PubMed
Google Scholar
Kobayashi A, Mizukoshi K, Iwasaki Y, Miyata H, Yoshida Y, Kitamoto T: Co-occurrence of types 1 and 2 PrPres in sporadic Creutzfeldt-Jakob disease MM1. Am J Pathol 2011, 178: 1309–1315.
Article
PubMed Central
CAS
PubMed
Google Scholar
Kobayashi A, Asano M, Mohri S, Kitamoto T: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain. J Biol Chem 2007, 282: 30022–30028.
Article
CAS
PubMed
Google Scholar
Paxinos G, Franklin KBJ: The mouse brain in stereotaxic coordinates. 4th edition. London: Academic Press; 2012.
Google Scholar
Kobayashi A, Asano M, Mohri S, Kitamoto T: A traceback phenomenon can reveal the origin of prion infection. Neuropathology 2009, 29: 619–624.
Article
PubMed
Google Scholar
Kretzschmar HA, Sethi S, Földvári Z, Windl O, Querner V, Zerr I, et al.: Iatrogenic Creutzfeldt-Jakob disease with florid plaques. Brain Pathol 2003, 13: 245–249.
Article
PubMed
Google Scholar
Doh-ura K, Kitamoto T, Sakaki Y, Tateishi J: CJD discrepancy. Nature 1991, 353: 801–802.
Article
CAS
PubMed
Google Scholar
Hizume M, Kobayashi A, Teruya K, Ohashi H, Ironside JW, Mohri S, et al.: Human prion protein (PrP) 219K is converted to PrPSc but shows heterozygous inhibition in variant Creutzfeldt-Jakob disease infection
. J Biol Chem 2009, 284: 3603–3609.
Article
CAS
PubMed
Google Scholar
Kobayashi A, Hizume M, Teruya K, Mohri S, Kitamoto T: Heterozygous inhibition in prion infection: the stone fence model
. Prion 2009, 3: 27–30.
Article
PubMed Central
CAS
PubMed
Google Scholar
Parchi P, Cescatti M, Notari S, Schulz-Schaeffer WJ, Capellari S, Giese A, et al.: 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 2010, 133: 3030–3042.
Article
PubMed Central
PubMed
Google Scholar
Yamada M, Noguchi-Shinohara M, Hamaguchi T, Nozaki I, Kitamoto T, Sato T, et al.: Dura mater graft-associated Creutzfeldt-Jakob disease in Japan: clinicopathological and molecular characterization of the two distinct subtypes. Neuropathology 2009, 29: 609–618.
Article
PubMed
Google Scholar