Synthetic Mammalian Prions

Giuseppe Legname; Ilia V. Baskakov; Hoang-Oanh B. Nguyen; Detlev Riesner; Fred E. Cohen; Stephen J. DeArmond; Stanley B. Prusiner

doi:10.1126/science.1100195

Abstract

Recombinant mouse prion protein (recMoPrP) produced in Escherichia coli was polymerized into amyloid fibrils that represent a subset of β sheet–rich structures. Fibrils consisting of recMoPrP(89–230) were inoculated intracerebrally into transgenic (Tg) mice expressing MoPrP(89–231). The mice developed neurologic dysfunction between 380 and 660 days after inoculation. Brain extracts showed protease-resistant PrP by Western blotting; these extracts transmitted disease to wild-type FVB mice and Tg mice overexpressing PrP, with incubation times of 150 and 90 days, respectively. Neuropathological findings suggest that a novel prion strain was created. Our results provide compelling evidence that prions are infectious proteins.

Keywords

Recombinant DNAGenetically modified mouseEscherichia coliBlotFibrilPrion proteinInoculationIncubationTransgeneMolecular biologyVirologyIncubation periodStrain (injury)BiologyChemistryBiochemistryPathologyImmunologyDiseaseMedicineGeneAnatomy

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Publication Info

Year: 2004
Type: article
Volume: 305
Issue: 5684
Pages: 673-676
Citations: 1013
Access: Closed

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APA Style

                            
                                
                                    Giuseppe Legname, 
                                
                                    Ilia V. Baskakov, 
                                
                                    Hoang-Oanh B. Nguyen
                                
                                et al.
                            
                            (2004). 
                            Synthetic Mammalian Prions. 
                            Science
                            , 305
                            (5684)
                            , 673-676.
                            https://doi.org/10.1126/science.1100195
                        

Identifiers

DOI: 10.1126/science.1100195