<?xml version="1.0" encoding="UTF-8"?><xml><records><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Nuvolone, M.</style></author><author><style face="normal" font="default" size="100%">Hermann, M.</style></author><author><style face="normal" font="default" size="100%">Sorce, S.</style></author><author><style face="normal" font="default" size="100%">Russo, G.</style></author><author><style face="normal" font="default" size="100%">Tiberi, C.</style></author><author><style face="normal" font="default" size="100%">Schwarz, P.</style></author><author><style face="normal" font="default" size="100%">Minikel, E.</style></author><author><style face="normal" font="default" size="100%">Sanoudou, D.</style></author><author><style face="normal" font="default" size="100%">Pelczar, P.</style></author><author><style face="normal" font="default" size="100%">Aguzzi, A.</style></author></authors></contributors><titles><title><style face="normal" font="default" size="100%">Strictly co-isogenic C57BL/6J-Prnp-/- mice: A rigorous resource for prion science</style></title><secondary-title><style face="normal" font="default" size="100%">J Exp MedJ Exp MedJ Exp Med</style></secondary-title><alt-title><style face="normal" font="default" size="100%">The Journal of experimental medicine</style></alt-title><short-title><style face="normal" font="default" size="100%">The Journal of experimental medicineThe Journal of experimental medicine</style></short-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">Animals</style></keyword><keyword><style  face="normal" font="default" size="100%">Base Sequence</style></keyword><keyword><style  face="normal" font="default" size="100%">Chromosomes, Mammalian</style></keyword><keyword><style  face="normal" font="default" size="100%">Endonucleases/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Female</style></keyword><keyword><style  face="normal" font="default" size="100%">Gene Deletion</style></keyword><keyword><style  face="normal" font="default" size="100%">Macrophages/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Male</style></keyword><keyword><style  face="normal" font="default" size="100%">Mice, Inbred C57BL</style></keyword><keyword><style  face="normal" font="default" size="100%">Molecular Sequence Data</style></keyword><keyword><style  face="normal" font="default" size="100%">Nerve Degeneration/pathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Open Reading Frames/genetics</style></keyword><keyword><style  face="normal" font="default" size="100%">Phagocytosis</style></keyword><keyword><style  face="normal" font="default" size="100%">Phenotype</style></keyword><keyword><style  face="normal" font="default" size="100%">Polyradiculoneuropathy/pathology</style></keyword><keyword><style  face="normal" font="default" size="100%">Prions/*metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">RNA/metabolism</style></keyword><keyword><style  face="normal" font="default" size="100%">Sequence Analysis, RNA</style></keyword><keyword><style  face="normal" font="default" size="100%">Trans-Activators/genetics</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2016</style></year><pub-dates><date><style  face="normal" font="default" size="100%">Mar 7</style></date></pub-dates></dates><number><style face="normal" font="default" size="100%">3</style></number><volume><style face="normal" font="default" size="100%">213</style></volume><pages><style face="normal" font="default" size="100%">313-27</style></pages><isbn><style face="normal" font="default" size="100%">1540-9538 (Electronic)0022-1007 (Linking)</style></isbn><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">Although its involvement in prion replication and neurotoxicity during transmissible spongiform encephalopathies is undisputed, the physiological role of the cellular prion protein (PrP(C)) remains enigmatic. A plethora of functions have been ascribed to PrP(C) based on phenotypes of Prnp(-/-) mice. However, all currently available Prnp(-/-) lines were generated in embryonic stem cells from the 129 strain of the laboratory mouse and mostly crossed to non-129 strains. Therefore, Prnp-linked loci polymorphic between 129 and the backcrossing strain resulted in systematic genetic confounders and led to erroneous conclusions. We used TALEN-mediated genome editing in fertilized mouse oocytes to create the Zurich-3 (ZH3) Prnp-ablated allele on a pure C57BL/6J genetic background. Genomic, transcriptional, and phenotypic characterization of Prnp(ZH3/ZH3) mice failed to identify phenotypes previously described in non-co-isogenic Prnp(-/-) mice. However, aged Prnp(ZH3/ZH3) mice developed a chronic demyelinating peripheral neuropathy, confirming the crucial involvement of PrP(C) in peripheral myelin maintenance. This new line represents a rigorous genetic resource for studying the role of PrP(C) in physiology and disease.</style></abstract><accession-num><style face="normal" font="default" size="100%">26926995</style></accession-num><notes><style face="normal" font="default" size="100%">Nuvolone, MarioHermann, MarioSorce, SilviaRusso, GiancarloTiberi, CinziaSchwarz, PetraMinikel, EricSanoudou, DespinaPelczar, PawelAguzzi, AdrianoengResearch Support, Non-U.S. Gov't2016/03/02 06:00J Exp Med. 2016 Mar 7;213(3):313-27. doi: 10.1084/jem.20151610. Epub 2016 Feb 29.</style></notes><custom2><style face="normal" font="default" size="100%">4813672</style></custom2><auth-address><style face="normal" font="default" size="100%">Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland.Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland Institute of Laboratory Animal Science, University of Zurich, 8091 Zurich, Switzerland.Functional Genomics Center Zurich (FGCZ), 8057 Zurich, Switzerland.Prion Alliance, Cambridge, MA 02139 Broad Institute, Cambridge, MA 02142 Analytical and Translational Genetics Unit, Massachusetts General Hospital, Boston, MA 02114.Fourth Department of Internal Medicine, Attikon Hospital, Medical School, University of Athens, 115 27 Athens, Greece.Institute of Laboratory Animal Science, University of Zurich, 8091 Zurich, Switzerland.Institute of Neuropathology, University Hospital of Zurich, 8091 Zurich, Switzerland adriano.aguzzi@usz.ch.</style></auth-address></record></records></xml>