Transgenic Fatal Familial Insomnia Mice Indicate Prion Infectivity-Independent Mechanisms of Pathogenesis and Phenotypic Expression of Disease

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Bouybayoune, I.; Mantovani, S.; Del Gallo, F.; Bertani, I.; Restelli, E.; Comerio, L.; Tapella, L.; Baracchi, F.; Fernández-Borges, N.; Mangieri, M.; Bisighini, C.; Beznoussenko, G..V.; Paladini, A.; Balducci, C.; Micotti, E.; Forloni, G.; Castilla, J.; Fiordaliso, F.; Tagliavini, F.; Imeri, L.; Chiesa, R.;
  • Publisher: Public Library of Science
  • Journal: PLoS Pathogens,volume 11,issue 4 (issn: 1553-7366, eissn: 1553-7374)
  • Publisher copyright policies & self-archiving
  • Related identifiers: doi: 10.1371/journal.ppat.1004796, pmc: PMC4400166
  • Subject: RC581-607 | Research Article | sleep-wake cycle; prion protein; prion disorders | Settore BIO/09 - Fisiologia | Immunologic diseases. Allergy | Biology (General) | QH301-705.5
    mesheuropmc: nervous system diseases | animal diseases | mental disorders

Author Summary Genetic prion diseases are degenerative brain disorders caused by mutations in the gene encoding the prion protein (PrP). Different PrP mutations cause different diseases, including Creutzfeldt-Jakob disease (CJD) and fatal familial insomnia (FFI). The re... View more
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