multiorgan detection and characterization of protease-resistant prion protein in a case of variant cjd examined in the united statesmultiorgan protease-resistant朊病毒蛋白的检测和表征变异型克雅氏病的检查在美国.pdfVIP

Multiorgan Detection and Characterization of Protease- Resistant Prion Protein in a Case of Variant CJD Examined in the United States 1. 1. 2 3 3 Silvio Notari , Francisco J. Moleres , Stephen B. Hunter , Ermias D. Belay , Lawrence B. Schonberger , 1 4 3 5 3 1 Ignazio Cali , Piero Parchi , Wun-Ju Shieh , Paul Brown , Sherif Zaki , Wen-Quan Zou , Pierluigi Gambetti1* 1 Institute of Pathology, Case Western Reserve University, Cleveland, Ohio, United States of America, 2 Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, Georgia, United States of America, 3 National Center for Zoonotic, Vector-Borne, and Enteric Diseases, Center for Disease Control and Prevention, Atlanta, Georgia, United States of America, 4 Dipartimento di Scienze Neurologiche, Universita’ di Bologna, Italy, 5 CEA/DSV/iMETI/SEPIA, France Abstract Background: Variant Creutzfeldt–Jakob disease (vCJD) is a prion disease thought to be acquired by the consumption of prion-contaminated beef products. To date, over 200 cases have been identified around the world, but mainly in the United Kingdom. Three cases have been identified in the United States; however, these subjects were likely exposed to prion infection elsewhere. Here we report on the first of these subjects. Methodology/Principal Findings: Neuropathological and genetic examinations were carried out using standard procedures. We assessed the presence and characteristics of protease-resistant prion protein (PrPres) in brain and 23 other or