Ultrasensitive method for the diagnosis of prion diseases (Creutzfeldt-Jakob disease and BSE)

German scientists have developed a novel, highly sensitive technique for the detection of prions, the infectious agents of Creutzfeldt-Jakob disease (CJD) and BSE. By this method, single prion particles can be identified in body fluids. For the first time, prions could be detected in the cerebrospinal fluid (CSF) of CJD patients. Currently, a clinical test to be used on a routine basis is being developed. (J. Bieschke, A. Giese, W. Schulz-Schaeffer, I. Zerr, S. Poser, M. Eigen, H. Kretzschmar: "Ultra-sensitive detection of pathological prion protein aggregates by dual-color scanning for intensely fluorescent targets" Proc. Natl. Acad. Sci. USA (2000) 97:5468-5473)

Creutzfeldt-Jakob disease in humans and BSE in cattle belong to a group of transmissible and invariably fatal diseases of the central nervous system. They are caused by unusual infectious agents known as prions. Prions lack a detectable genome. They consist of a pathological aggregated form of a host-derived protein called prion protein. The definite diagnosis of prion disease is based on the demonstration of pathological prion protein accumulation in brain tissue, however, suitable clinical tests for the detection of prions in living patients or animals have not been available.

A novel diagnostic technique now exists that permits direct detection of prions in the cerebrospinal fluid, which is easily accessible for diagnostic purposes. Supported by the German Federal Ministry of Research, this method was developed in a cooperative effort by two research groups, that of the Nobel laureate, Manfred Eigen of the Max-Planck Institute of Biophysical Chemistry in Gttingen, and that of the prion researcher, Prof. Hans Kretzschmar of the University of Gttingen (now at the University of Munich).

This technique is based on a setup designed for confocal, dual-color fluorescence cross-correlation spectroscopy. Following excitation with a highly focussed laser beam, this setup permits the detection o

Contact: Dr. Jan Bieschke, Dr. Armin Giese
jbiesch@gwdg.de, agiese@med.uni-goettingen.de

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