The DOI matches its Crossref record; 42 of the 42 references it cites were found in a registry or an index.
Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification
Open accessAbstract
Six subtypes of sporadic Creutzfeldt-Jakob disease with distinctive clinico-pathological features have been identified largely based on two types of the abnormal prion protein, PrP(Sc), and the methionine (M)/valine (V) polymorphic codon 129 of the prion protein. The existence of affected subjects showing mixed phenotypic features and concurrent PrP(Sc) types has been reported but with inconsistencies among studies in both results and their interpretation. The issue currently complicates diagnosis and classification of cases and also has implications for disease pathogenesis. To explore the issue in depth, we carried out a systematic regional study in a large series of 225 cases. PrP(Sc) types 1 and 2 concurrence was detected in 35% of cases and was higher in MM than in MV or VV subjects. The deposition of either type 1 or 2, when concurrent, was not random and always characterized by the coexistence of phenotypic features previously described in the pure subtypes. PrP(Sc) type 1 accumulation and related pathology predominated in MM and MV cases, while the type 2 phenotype prevailed in VVs. Neuropathological examination best identified the mixed types 1 and 2 features in MMs and most MVs, and also uniquely revealed the co-occurrence of pathological variants sharing PrP(Sc) type 2. In contrast, molecular typing best detected the concurrent PrP(Sc) types in VV subjects and MV cases with kuru plaques. The present data provide an updated disease classification and are of importance for future epidemiologic and transmission studies aimed to identify etiology and extent of strain variation in sporadic Creutzfeldt-Jakob disease.
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Cites
Prusiner, S. B. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.23.13363 · Cited by 5,373
Pan, K. M., Baldwin, M., Nguyen, J., Gasset, M., Serban, A., Groth, D., Mehlhorn, I., Huang, Z., Fletterick, R. J., & Cohen, F. E. (1993) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.90.23.10962 · Cited by 2,277
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J., & Hill, A. F. (1996) · Nature · doi:10.1038/383685a0 · Cited by 1,735
Identification of a Protein That Purifies with the Scrapie Prion
Bolton, D. C., McKinley, M. P., & Prusiner, S. B. (1982) · Science · doi:10.1126/science.6815801 · Cited by 1,280
Eight prion strains have PrPSc molecules with different conformations
Safar, J., Wille, H., Itri, V., Groth, D., Serban, H., Torchia, M., Cohen, F. E., & Prusiner, S. B. (1998) · Nature Medicine · doi:10.1038/2654 · Cited by 1,245
Telling, G. C., Parchi, P., DeArmond, S. J., Cortelli, P., Montagna, P., Gabizon, R., Mastrianni, J., Lugaresi, E., Gambetti, P., & Prusiner, S. B. (1996) · Science · doi:10.1126/science.274.5295.2079 · Cited by 865
Self-citationMolecular basis of phenotypic variability in sporadc creudeldt‐jakob disease
Parchi, P., Castellani, R., Capellari, S., Ghetti, B., Young, K., Chen, S. G., Farlow, M., Dickson, D. W., Sima, A. A. F., Trojanowski, J. Q., et al. (1996) · Annals of Neurology · doi:10.1002/ana.410390613 · Cited by 864
Self-citationCaughey, B. W., Dong, A., Bhat, K. S., Ernst, D., Hayes, S. F., & Caughey, W. S. (1991) · Biochemistry · doi:10.1021/bi00245a003 · Cited by 822
Bessen, R. A., & Marsh, R. F. (1994) · Journal of Virology · doi:10.1128/jvi.68.12.7859-7868.1994 · Cited by 665
Goldmann, W., Hunter, N., Smith, G., Foster, J., & Hope, J. (1994) · Journal of General Virology · doi:10.1099/0022-1317-75-5-989 · Cited by 431
Cited by
Rossi, M., Candelise, N., Baiardi, S., Capellari, S., Giannini, G., Orrù, C. D., Antelmi, E., Mammana, A., Hughson, A. G., Calandra-Buonaura, G., et al. (2020) · Acta Neuropathologica · doi:10.1007/s00401-020-02160-8 · Cited by 451
Self-citationThe prion hypothesis: from biological anomaly to basic regulatory mechanism
Tuite, M. F., & Serio, T. R. (2010) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3007 · Cited by 146
Kim, M.-O., Takada, L. T., Wong, K., Forner, S. A., & Geschwind, M. D. (2018) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a033134 · Cited by 130
Subtype and Regional-Specific Neuroinflammation in Sporadic Creutzfeldt–Jakob Disease
Llorens, F., López-González, I., Thüne, K., Carmona, M., Zafar, S., Andréoletti, O., Zerr, I., & Ferrer, I. (2014) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2014.00198 · Cited by 69
Self-citationSporadic Fatal Insomnia in Europe: Phenotypic Features and Diagnostic Challenges
Abu‐Rumeileh, S., Redaelli, V., Baiardi, S., Mackenzie, G., Windl, O., Ritchie, D. L., Didato, G., Hernandez‐Vara, J., Rossi, M., Capellari, S., et al. (2018) · Annals of Neurology · doi:10.1002/ana.25300 · Cited by 66
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Metadata
- Authors
- Parchi, P., Strammiello, R., Notari, S., Giese, A., Langeveld, J. P. M., Ladogana, A., Zerr, I., Roncaroli, F., Cras, P., Ghetti, B., Pocchiari, M., Kretzschmar, H., & Capellari, S.
- Venue
- Acta Neuropathologica
- Published
- 2009-08-29
- Volume
- 118
- Issue
- 5
- Pages
- 659-671
- ISSN
- 0001-6322 (ISSN-L)
- 1432-0533
- OpenAlex
- W2137802088
- Cited by
- 263
- References
- 42
- FWCI
- 6.12
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Parchi, P., Strammiello, R., Notari, S., Giese, A., Langeveld, J. P. M., Ladogana, A., Zerr, I., Roncaroli, F., Cras, P., Ghetti, B., Pocchiari, M., Kretzschmar, H., & Capellari, S. (2009). Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathologica. https://doi.org/10.1007/s00401-009-0585-1
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