Quick Search:

uzh logo
Browse by:
bullet
bullet
bullet
bullet

Zurich Open Repository and Archive

Permanent URL to this publication: http://dx.doi.org/10.5167/uzh-1884

Glatzel, M; Abela, E; Maissen, M; Aguzzi, A (2003). Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease. New England Journal of Medicine, 349(19):1812-1820.

[img]
Preview
PDF
281kB

View at publisher

Abstract

BACKGROUND: In patients with sporadic Creutzfeldt-Jakob disease, pathologic disease-associated prion protein (PrPSc) has been identified only in the central nervous system and olfactory-nerve tissue. Understanding the distribution of PrPSc in Creutzfeldt-Jakob disease is important for classification and diagnosis and perhaps even for prevention. METHODS: We used a highly sensitive method of detection--involving the concentration of PrPSc by differential precipitation with sodium phosphotungstic acid, which increased the sensitivity of Western blot analysis by up to three orders of magnitude--to search for PrPSc in extraneural organs of 36 patients with sporadic Creutzfeldt-Jakob disease who died between 1996 and 2002. RESULTS: PrPSc was present in the brain tissue of all patients. In addition, we found PrPSc in 10 of 28 spleen specimens and in 8 of 32 skeletal-muscle samples. Three patients had PrPSc in both spleen and muscle specimens. Patients with extraneural PrPSc had a significantly longer duration of disease and were more likely to have uncommon molecular variants of sporadic Creutzfeldt-Jakob disease than were patients without extraneural PrPSc. CONCLUSIONS: Using sensitive techniques, we identified extraneural deposition of PrPSc in spleen and muscle samples from approximately one third of patients who died with sporadic Creutzfeldt-Jakob disease. Extraneural PrPSc appears to correlate with a long duration of disease.

Citations

206 citations in Web of Science®
220 citations in Scopus®
Google Scholar™

Altmetrics

Downloads

48 downloads since deposited on 11 Feb 2008
9 downloads since 12 months

Detailed statistics

Additional indexing

Item Type:Journal Article, refereed
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
DDC:570 Life sciences; biology
610 Medicine & health
Language:English
Date:6 November 2003
Deposited On:11 Feb 2008 12:26
Last Modified:27 Nov 2013 18:13
Publisher:Massachusetts Medical Society
ISSN:0028-4793
Publisher DOI:10.1056/NEJMoa030351
PubMed ID:14602879

Users (please log in): suggest update or correction for this item

Repository Staff Only: item control page