Transmission of prion disease by blood and hot news in detection methods of abnormal prion protein

Overview

Prion diseases, so called transmissible spongiform encephalopathies (TSE), are neurodegenerative diseases, which affect both human and animals. Around 85% of human prion diseases is sporadic, 10–15% is inherited and 2–3% is acquired. The most frequent one is sporadic Creutzfeldt-Jakob disease (sCJD). As yet there is no treatment available, moreover we are not able to rapidly detect prion protein ante mortem. In England two cases of variant Creutzfeldt-Jakob disease (vCJD) transmitted by blood have been reported recently. vCJD rises due to exposure to bovine spongiform encephalopathy (BSE) contaminated food. There has been no case of vCJD in Czech Republic yet, however, despite preventive measures 20 cases of BSE have been already detected. Therefore, we can not exclude incidence of vCJD in Czech Republic. Such a case is a threat for transfusion medicine, as there is no available screening test. This article is dedicated to vCJD and transmission of this disease by blood. Hot topics from diagnostic field are presented at the end.

Key words:
prion protein, PrPsc, Creutzfeldt-Jakob disease, CJD, preclinical detection