Comparison of the level, distribution and form of disease-associated prion protein in variant and sporadic Creutzfeldt-Jakob diseased brain using conformation-dependent immunoassay and Western blot

Journal of General Virology

Volumn 92, Issue 3, 2011, Pages 727-732

  Choi, Young Pyo ^a   Gröner, Albrecht ^b   Ironside, James W ^a   Head, Mark W ^a  

^a UNIVERSITY OF EDINBURGH   (United Kingdom)

^b CSL BEHRING GMBH   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN; PROTEINASE;

ARTICLE; BRAIN; BRAIN REGION; CONFORMATION DEPENDENT IMMUNOASSAY; CONTROLLED STUDY; DIAGNOSTIC TEST ACCURACY STUDY; DISEASE PREDISPOSITION; GENETIC VARIABILITY; HUMAN; HUMAN TISSUE; IMMUNOASSAY; PRION; PRIORITY JOURNAL; PROTEIN CONFORMATION; PROTEIN DEGRADATION; PROTEIN LOCALIZATION; PROTEIN TARGETING; SENSITIVITY AND SPECIFICITY; SPORADIC CREUTZFELDT JAKOB DISEASE; VIRUS DETECTION; WESTERN BLOTTING;

BRAIN; CREUTZFELDT-JAKOB SYNDROME; HUMANS; IMMUNOASSAY; PRIONS;

EID: 79951802675     PISSN: 00221317     EISSN: 14652099     Source Type: Journal    
DOI: 10.1099/vir.0.026948-0     Document Type: Article

References (30)

1. Bellon, A., Seyfert-Brandt, W., Lang, W., Baron, H., Gröner, A. & Vey, M. (2003). Improved conformation-dependent immunoassay: suitability for human prion detection with enhanced sensitivity. J Gen Virol 84, 1921-1925. (Pubitemid 36805928)
2. Bolton, D. C., McKinley, M. P. & Prusiner, S. B. (1982). Identification of a protein that purifies with the scrapie prion. Science 218, 1309-1311.
3. Caughey, B. W., Dong, A., Bhat, K. S., Ernst, D., Hayes, S. F. & Caughey, W. S. (1991). Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy. Biochemistry 30, 7672-7680.
4. Colby, D. W., Wain, R., Baskakov, I. V., Legname, G., Palmer, C. G., Nguyen, H. O., Lemus, A., Cohen, F. E., DeArmond, S. J. & Prusiner, S. B. (2010). Protease-sensitive synthetic prions. PLoS Pathog 6, e1000736.
5. Collinge, J., Sidle, K. C., Meads, J., Ironside, J. & Hill, A. F. (1996). Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 383, 685-690.
6. Cronier, S., Gros, N., Tattum, M. H., Jackson, G. S., Clarke, A. R., Collinge, J. & Wadsworth, J. D. (2008). Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin. Biochem J 416, 297-305.
7. Deleault, A. M., Deleault, N. R., Harris, B. T., Rees, J. R. & Supattapone, S. (2008). The effects of prion protein proteolysis and disaggregation on the strain properties of hamster scrapie. J Gen Virol 89, 2642-2650.
8. Gambetti, P., Kong, Q., Zou, W., Parchi, P. & Chen, S. G. (2003). Sporadic and familial CJD: classification and characterisation. Br Med Bull 66, 213-239. (Pubitemid 37173504)
9. Gambetti, P., Dong, Z., Yuan, J., Xiao, X., Zheng, M., Alshekhlee, A., Castellani, R., Cohen, M., Barria, M. A. & other authors (2008). A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 63, 697-708. (Pubitemid 351945529)
10. Head, M. W., Bunn, T. J., Bishop, M. T., McLoughlin, V., Lowrie, S., McKimmie, C. S., Williams, M. C., McCardle, L., MacKenzie, J. & other authors (2004). Prion protein heterogeneity in sporadic but not variant Creutzfeldt-Jakob disease: UK cases 1991-2002. Ann Neurol 55, 851-859.
11. Meyer, R. K., McKinley, M. P., Bowman, K. A., Braunfeld, M. B., Barry, R. A. & Prusiner, S. B. (1986). Separation and properties of cellular and scrapie prion proteins. Proc Natl Acad Sci U S A 83, 2310-2314. (Pubitemid 16026876)
12. Notari, S., Strammiello, R., Capellari, S., Giese, A., Cescatti, M., Grassi, J., Ghetti, B., Langeveld, J. P., Zou, W. Q. & other authors (2008). Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem 283, 30557-30565.
13. Oesch, B., Westaway, D., Wälchli, M., McKinley, M. P., Kent, S. B., Aebersold, R., Barry, R. A., Tempst, P., Teplow, D. B. & other authors (1985). A cellular gene encodes scrapie PrP 27-30 protein. Cell 40, 735-746. (Pubitemid 15240279)
14. Pan, K. M., Baldwin, M., Nguyen, J., Gasset, M., Serban, A., Groth, D., Mehlhorn, I., Huang, Z., Fletterick, R. J. & other authors (1993). Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins. Proc Natl Acad Sci U S A 90, 10962-10966.
15. Parchi, P., Castellani, R., Capellari, S., Ghetti, B., Young, K., Chen, S. G., Farlow, M., Dickson, D. W., Sima, A. A. & other authors (1996). Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 39, 767-778. (Pubitemid 26183813)
16. Parchi, P., Giese, A., Capellari, S., Brown, P., Schulz-Schaeffer, W., Windl, O., Zerr, I., Budka, H., Kopp, N. & other authors (1999). Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46, 224-233. (Pubitemid 29382879)
17. Peretz, D., Williamson, R. A., Matsunaga, Y., Serban, H., Pinilla, C., Bastidas, R. B., Rozenshteyn, R., James, T. L., Houghten, R. A. & other authors (1997). A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. J Mol Biol 273, 614-622.
18. Prusiner, S. B. (1982). Novel proteinaceous infectious particles cause scrapie. Science 216, 136-144.
19. Prusiner, S. B. (1998). Prions. Proc Natl Acad Sci U S A 95, 13363-13383.
20. Ritchie, D. L., Boyle, A., McConnell, I., Head, M. W., Ironside, J. W. & Bruce, M. E. (2009). Transmissions of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice. J Gen Virol 90, 3075-3082.
21. Sc molecules with different conformations. Nat Med 4, 1157-1165.
22. Safar, J. G., Scott, M., Monaghan, J., Deering, C., Didorenko, S., Vergara, J., Ball, H., Legname, G., Leclerc, E. & other authors (2002). Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice. Nat Biotechnol 20, 1147-1150. (Pubitemid 35285445)
23. Safar, J. G., Geschwind, M. D., Deering, C., Didorenko, S., Sattavat, M., Sanchez, H., Serban, A., Vey, M., Baron, H. & other authors (2005). Diagnosis of human prion disease. Proc Natl Acad Sci U S A 102, 3501-3506. (Pubitemid 40328074)
24. Sc profiling in sporadic Creutzfeldt-Jakob disease. PLoS Med 3, e14.
25. Thackray, A. M., Hopkins, L. & Bujdoso, R. (2007). Proteinase K-sensitive disease-associated ovine prion protein revealed by conformation-dependent immunoassay. Biochem J 401, 475-483. (Pubitemid 46166430)
26. Tremblay, P., Ball, H. L., Kaneko, K., Groth, D., Hegde, R. S., Cohen, F. E., DeArmond, S. J., Prusiner, S. B. & Safar, J. G. (2004). Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol 78, 2088-2099. (Pubitemid 38176741)
27. Tzaban, S., Friedlander, G., Schonberger,O., Horonchik, L., Yedidia, Y., Shaked, G., Gabizon, R. & Taraboulos, A. (2002). Protease-sensitive scrapie prion protein in aggregates of heterogeneous sizes. Biochemistry 41, 12868-12875. (Pubitemid 35192516)
28. Wadsworth, J. D., Joiner, S., Hill, A. F., Campbell, T. A., Desbruslais, M., Luthert, P. J. & Collinge, J. (2001). Tissue distribution of protease resistant prion protein in variant Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 358, 171-180.
29. Yull, H. M., Ritchie, D. L., Langeveld, J. P., van Zijderveld, F. G., Bruce, M. E., Ironside, J. W. & Head, M. W. (2006). Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol 168, 151-157. (Pubitemid 43062568)
30. Yull, H. M., Ironside, J. W. & Head, M. W. (2009). Further characterisation of the prion protein molecular types detectable in the NIBSC Creutzfeldt-Jakob disease brain reference materials. Biologicals 37, 210-215.