Original Paper

Association between Deposition of Beta-Amyloid and Pathological Prion Protein in Sporadic Creutzfeldt-Jakob Disease
Laura Debatin^a, Johannes Streffer^b, Markus Geissen^c, Jakob Matschke^c, Adriano Aguzzi^a, Markus Glatzel<sup>a, c

a</sup>Institute of Neuropathology, and
^bDivision of Psychiatry Research, University Hospital Zurich, Zurich, Switzerland;
^cInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

Address of Corresponding Author

Neurodegenerative Dis 2008;5:347-354 (DOI: 10.1159/000121389)

  Key Words

• Sporadic Creutzfeldt-Jakob disease
• Alzheimer's disease
• Deposition of -amyloid
• Prion protein

  Abstract

Background: Alzheimer's disease (AD) and prion diseases such as sporadic Creutzfeldt-Jakob disease (sCJD) share common features concerning their molecular pathogenesis and neuropathological presentation and the coexistence of AD and CJD in patients suggest an association between the deposition of the proteolytically processed form of the amyloid precursor protein, -amyloid (A), which deposits in AD, and the abnormal form of the prion protein, PrP^Sc, which deposits in sCJD. Methods: We have characterized sCJD patients (n = 14), AD patients (n = 5) and nondemented controls (n = 5) with respect to the deposition of PrP^Sc and A morphologically, biochemically and genetically and correlated these findings to clinical data. Results: sCJD-diseased individuals with abundant deposits of A present with a specific clinicopathological profile, defined by higher age at disease onset, long disease duration, a genetic profile and only minimal amounts of PrP^Sc in the cerebellum. Conclusion: The co-occurrence of pathological changes typical for sCJD and AD in combination with the inverse association between accumulation of A and PrP^Sc in a subgroup of sCJD patients is indicative of common pathways involved in the generation or clearance of A and PrP^Sc in a subgroup of sCJD patients.

Copyright © 2008 S. Karger AG, Basel

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  Author Contacts

Markus Glatzel
Institute of Neuropathology, University Medical Center Hamburg-Eppendorf
Martinistrasse 52, DE-20246 Hamburg (Germany)
Tel. +49 40 42 803 2218, Fax +49 40 42 803 4929
E-Mail m.glatzel@uke.uni-hamburg.de

  Article Information

L.D. and J.S. contributed equally to this work.

Received: May 7, 2007
Accepted after revision: October 31, 2007
Published online: March 18, 2008
Number of Print Pages : 8
Number of Figures : 3, Number of Tables : 1, Number of References : 43

  Publication Details

Neurodegenerative Diseases

Vol. 5, No. 6, Year 2008 (Cover Date: September 2008)

Journal Editor: Nitsch R.M. (Zürich), Hock C. (Zürich)
ISSN: 1660-2854 (Print), eISSN: 1660-2862 (Online)

For additional information: http://www.karger.com/NDD

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