@article {26573,
	title = {Strictly co-isogenic C57BL/6J-Prnp-/- mice: A rigorous resource for prion science},
	journal = {J Exp MedJ Exp MedJ Exp Med},
	volume = {213},
	number = {3},
	year = {2016},
	note = {Nuvolone, MarioHermann, MarioSorce, SilviaRusso, GiancarloTiberi, CinziaSchwarz, PetraMinikel, EricSanoudou, DespinaPelczar, PawelAguzzi, AdrianoengResearch Support, Non-U.S. Gov{\textquoteright}t2016/03/02 06:00J Exp Med. 2016 Mar 7;213(3):313-27. doi: 10.1084/jem.20151610. Epub 2016 Feb 29.},
	month = {Mar 7},
	pages = {313-27},
	abstract = {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.},
	keywords = {Animals, Base Sequence, Chromosomes, Mammalian, Endonucleases/metabolism, Female, Gene Deletion, Macrophages/metabolism, Male, Mice, Inbred C57BL, Molecular Sequence Data, Nerve Degeneration/pathology, Open Reading Frames/genetics, Phagocytosis, Phenotype, Polyradiculoneuropathy/pathology, Prions/*metabolism, RNA/metabolism, Sequence Analysis, RNA, Trans-Activators/genetics},
	isbn = {1540-9538 (Electronic)0022-1007 (Linking)},
	author = {Nuvolone, M. and Hermann, M. and Sorce, S. and Russo, G. and Tiberi, C. and Schwarz, P. and Minikel, E. and Sanoudou, D. and Pelczar, P. and Aguzzi, A.}
}
