1 Title: Evaluation of the Human Transmission Risk of an Atypical Bovine Spongiform 1 Encephalopathy Prion Strain 2 Running Title: BASE Transmission in Humans 3 Authors: Qingzhong Kong, 1 * Mengjie Zheng, 1 Cristina Casalone, 2 Liuting Qing, 1 Shenghai 4 Huang, 1# Bikram Chakraborty, 1 Ping Wang, 1 Fusong Chen, 1 Ignazio Cali, 1 Cristiano Corona, 2 5 Francesca Martucci, 2 Barbara Iulini, 2 Pierluigi Acutis, 2 Lan Wang, 1 Jingjing Liang, 1 Meiling 6 Wang, 1 Xinyi Li, 1 Salvatore Monaco, 3 Gianluigi Zanusso, 3 Wen-Quan Zou, 1 Maria Caramelli, 2 7 Pierluigi Gambetti 1 * 8 1 Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 9 2 CEA, Istituto Zooprofilattico Sperimentale, 10154 Torino, Italy; 3 Department of Neurological 10 and Visual Sciences, University of Verona, 37134 Verona, Italy. # Current address: Department 11 of Patient Education and Health Information, Cleveland Clinic Foundation, Cleveland, OH 12 44195. 13 14 *Corresponding authors: 15 Pierluigi Gambetti, Department of Pathology, Case Western Reserve University, Cleveland, OH 16 44106, USA. Tel: 216-368-0586. Fax: 216-368-2546. Email: pxg13@case.edu . 17 & 18 Qingzhong Kong, Department of Pathology, Case Western Reserve University, Cleveland, OH 19 44106, USA. Tel: 216-368-1756. Fax: 216-368-2546. Email: qxk2@case.edu 20 21 Word counts: Abstract, 196 words; text, 2510 words. 22 ACCEPTED Copyright © 2008, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved. J. Virol. doi:10.1128/JVI.02561-07 JVI Accepts, published online ahead of print on 30 January 2008