JBC

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Originally published In Press as doi:10.1074/jbc.C100458200 on September 10, 2001

J. Biol. Chem., Vol. 276, Issue 44, 40377-40380, November 2, 2001
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow All Versions of this Article:
276/44/40377    most recent
C100458200v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Zanusso, G.
Right arrow Articles by Monaco, S.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Zanusso, G.
Right arrow Articles by Monaco, S.

ACCELERATED PUBLICATION
pH-dependent Prion Protein Conformation in Classical Creutzfeldt-Jakob Disease*

Gianluigi ZanussoDagger , Alessia FarinazzoDagger , Michele FioriniDagger , Matteo GelatiDagger , Annalisa Castagna§, Pier Giorgio Righetti§, Nicola RizzutoDagger , and Salvatore MonacoDagger ||

From the Dagger  Department of Neurological and Visual Sciences, University of Verona, Piazzale L. A. Scuro, 10, 37134 Verona, Italy and the § Department of Agricultural and Industrial Biotechnologies, University of Verona, Strada Le Grazie 15, 37134 Verona, Italy

In transmissible spongiform encephalopathies, the cellular prion protein (PrPC) undergoes a conformational change from a prevailing alpha -helical structure to a beta -sheet-rich, protease-resistant isoform, termed PrPSc. PrPC has two characteristics: a high affinity for Cu2+ and a strong pH-dependent conformation. Lines of evidence indicate that PrPSc conformation is dependent on copper and that acidic conditions facilitate the conversion of PrPC right-arrow PrPSc. In each species, PrPSc exists in multiple conformations, which are associated with different prion strains. In sporadic Creutzfeldt-Jakob disease (sCJD), different biochemical types of PrPSc have been identified according to the size of the protease-resistant fragments, patterns of glycosylation, and the metal-ion occupancy. Based on the site of cleavage produced by proteinase K, we investigated the conformational stability of PrPSc under acidic, neutral, and basic conditions in 42 sCJD subjects. Our study shows that only one type of sCJD PrPSc, associated with the classical form, shows a pH-dependent conformation, whereas two other biochemical PrPSc types, detected in distinct sCJD phenotypes, are unaffected by pH variations. This novel approach demonstrates the presence of three types of PrPSc in sCJD.

* This work was supported in part by Grant CNR CB00.00159.ST74.The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Supported by grants from Ministero dell'Università Scientifica e Tecnologica (coordinated project 40%, Proteome Analysis, 2000) and European Community (Contract No. QLRT-01903).

|| To whom correspondence should be addressed. Tel.: 39-045-8074286; Fax: 039-045-585933; E-mail: salvatore.monaco@mail. univr.it.

Copyright © 2001 by The American Society for Biochemistry and Molecular Biology, Inc.


This article has been cited by other articles:


Home page
 J. Virol.Home page
J. C. Espinosa, O. Andreoletti, J. Castilla, M. E. Herva, M. Morales, E. Alamillo, F. D. San-Segundo, C. Lacroux, S. Lugan, F. J. Salguero, et al.
Sheep-Passaged Bovine Spongiform Encephalopathy Agent Exhibits Altered Pathobiological Properties in Bovine-PrP Transgenic Mice
J. Virol., January 15, 2007; 81(2): 835 - 843.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
I. Cali, R. Castellani, J. Yuan, A. Al-Shekhlee, M. L. Cohen, X. Xiao, F. J. Moleres, P. Parchi, W.-Q. Zou, and P. Gambetti
Classification of sporadic Creutzfeldt-Jakob disease revisited.
Brain, September 1, 2006; 129(Pt 9): 2266 - 2277.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
A. F. Hill, S. Joiner, J. A. Beck, T. A. Campbell, A. Dickinson, M. Poulter, J. D. F. Wadsworth, and J. Collinge
Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations
Brain, March 1, 2006; 129(3): 676 - 685.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
V. Lewis, A. F. Hill, G. M. Klug, A. Boyd, C. L. Masters, and S. J. Collins
Australian sporadic CJD analysis supports endogenous determinants of molecular-clinical profiles
Neurology, July 12, 2005; 65(1): 113 - 118.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
S. G. Aller, E. T. Eng, C. J. De Feo, and V. M. Unger
Eukaryotic CTR Copper Uptake Transporters Require Two Faces of the Third Transmembrane Domain for Helix Packing, Oligomerization, and Function
J. Biol. Chem., December 17, 2004; 279(51): 53435 - 53441.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
G. Zanusso, A. Farinazzo, F. Prelli, M. Fiorini, M. Gelati, S. Ferrari, P. G. Righetti, N. Rizzuto, B. Frangione, and S. Monaco
Identification of Distinct N-terminal Truncated Forms of Prion Protein in Different Creutzfeldt-Jakob Disease Subtypes
J. Biol. Chem., September 10, 2004; 279(37): 38936 - 38942.
[Abstract] [Full Text] [PDF]

Home page
 Biophys. JHome page
L. Kreplak, J. Doucet, P. Dumas, and F. Briki
New Aspects of the {alpha}-Helix to {beta}-Sheet Transition in Stretched Hard {alpha}-Keratin Fibers
Biophys. J., July 1, 2004; 87(1): 640 - 647.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
S. Notari, S. Capellari, A. Giese, I. Westner, A. Baruzzi, B. Ghetti, P. Gambetti, H. A. Kretzschmar, and P. Parchi
Effects of Different Experimental Conditions on the PrPSc Core Generated by Protease Digestion: IMPLICATIONS FOR STRAIN TYPING AND MOLECULAR CLASSIFICATION OF CJD
J. Biol. Chem., April 16, 2004; 279(16): 16797 - 16804.
[Abstract] [Full Text] [PDF]

Home page
 J. Virol.Home page
S. Lezmi, S. Martin, S. Simon, E. Comoy, A. Bencsik, J.-P. Deslys, J. Grassi, M. Jeffrey, and T. Baron
Comparative Molecular Analysis of the Abnormal Prion Protein in Field Scrapie Cases and Experimental Bovine Spongiform Encephalopathy in Sheep by Use of Western Blotting and Immunohistochemical Methods
J. Virol., April 1, 2004; 78(7): 3654 - 3662.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
C. Casalone, G. Zanusso, P. Acutis, S. Ferrari, L. Capucci, F. Tagliavini, S. Monaco, and M. Caramelli
Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt-Jakob disease
PNAS, March 2, 2004; 101(9): 3065 - 3070.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Pathol.Home page
M. W. Head, D. Ritchie, N. Smith, V. McLoughlin, W. Nailon, S. Samad, S. Masson, M. Bishop, L. McCardle, and J. W. Ironside
Peripheral Tissue Involvement in Sporadic, Iatrogenic, and Variant Creutzfeldt-Jakob Disease: An Immunohistochemical, Quantitative, and Biochemical Study
Am. J. Pathol., January 1, 2004; 164(1): 143 - 153.
[Abstract] [Full Text] [PDF]

Home page
 J. Gen. Virol.Home page
G. Zanusso, C. Casalone, P. Acutis, E. Bozzetta, A. Farinazzo, M. Gelati, M. Fiorini, G. Forloni, M. S. Sy, S. Monaco, et al.
Molecular analysis of iatrogenic scrapie in Italy
J. Gen. Virol., April 1, 2003; 84(4): 1047 - 1052.
[Abstract] [Full Text] [PDF]

Home page
 NEJMHome page
G. Zanusso, S. Ferrari, F. Cardone, P. Zampieri, M. Gelati, M. Fiorini, A. Farinazzo, M. Gardiman, T. Cavallaro, M. Bentivoglio, et al.
Detection of Pathologic Prion Protein in the Olfactory Epithelium in Sporadic Creutzfeldt-Jakob Disease
N. Engl. J. Med., February 20, 2003; 348(8): 711 - 719.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
 All ASBMB Journals   Molecular and Cellular Proteomics 
 Journal of Lipid Research   ASBMB Today 
Copyright © 2001 by the American Society for Biochemistry and Molecular Biology.