Sporadic Creutzfeldt-Jakob disease: Co-occurrence of different types of PrPSc in the same brain

作者: G. Puoti , G. Giaccone , G. Rossi , B. Canciani , O. Bugiani

DOI: 10.1212/WNL.53.9.2173

关键词:

摘要: Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types the protease-resistant form prion protein (type 1 and type 2 PrP(Sc)). We investigated 14 cases CJD found that both PrP(Sc) coexisted in 5 subjects. The isoforms were associated with different patterns PrP deposition severity spongiform changes, suggesting plays a central role determining neuropathologic profile CJD.

参考文章(10)
Richard A. Bessen, David A. Kocisko, Gregory J. Raymond, Santosh Nandan, Peter T. Lansbury, Byron Caughey, Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature. ,vol. 375, pp. 698- 700 ,(1995) , 10.1038/375698A0
John Collinge, Andrew F. Hill, Katie C. L. Sidle, James Ironside, Biochemical typing of scrapie strains Nature. ,vol. 386, pp. 564- 564 ,(1997) , 10.1038/386564B0
Jiri Safar, Holger Wille, Vincenza Itri, Darlene Groth, Hana Serban, Marilyn Torchia, Fred E. Cohen, Stanley B. Prusiner, Eight prion strains have PrP Sc molecules with different conformations Nature Medicine. ,vol. 4, pp. 1157- 1165 ,(1998) , 10.1038/2654
Piero Parchi, Rudolph Castellani, Sabina Capellari, Bernardino Ghetti, Katherine Young, Shu G. Chen, Martin Farlow, Dennis W. Dickson, Anders A. F. Sima, John Q. Trojanowski, Robert B. Petersen, Pierluigi Gambetti, Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Annals of Neurology. ,vol. 39, pp. 767- 778 ,(1996) , 10.1002/ANA.410390613
John Collinge, Katie C. L. Sidle, Julie Meads, James Ironside, Andrew F. Hill, Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD Nature. ,vol. 383, pp. 685- 690 ,(1996) , 10.1038/383685A0
Piero Parchi, Armin Giese, Sabina Capellari, Paul Brown, Walter Schulz‐Schaeffer, Otto Windl, Inga Zerr, Herbert Budka, Nicolas Kopp, Pedro Piccardo, Sigrid Poser, Amyn Rojiani, Nathalie Streichemberger, Jean Julien, Claude Vital, Bernardino Ghetti, Pierluigi Gambetti, Hans Kretzschmar, None, Classification of sporadic Creutzfeldt‐Jakob disease based on molecular and phenotypic analysis of 300 subjects Annals of Neurology. ,vol. 46, pp. 224- 233 ,(1999) , 10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W
Stanley B. Prusiner, Prion Diseases and the BSE Crisis Science. ,vol. 278, pp. 245- 251 ,(1997) , 10.1126/SCIENCE.278.5336.245