Mastrianni JA, Capellari S, Telling GC, Han D, Bosque P, Prusiner SB, Dearmond SJ. Inherited prion disease caused by the V210I mutation: transmission to transgenic mice. Neurology. 2001 Dec 26;57(12):2198-205. PubMed Abstract

REAGENTS/MATERIAL:
Brain extracts from three human cases of familial Creutzfeldt–Jakob disease (fCJD) (V210I) were injected into transgenic (Tg) mice harboring only the chimeric mouse–human–mouse prion protein (PrP) (Tg[MHu2M]Prnp0/0) and produced disease in all mice by 200 days. Mouse brains were evaluated for PrP by Western blot with monoclonal anti-PrP (3F4) ab at a 1:5,000 dilution. For IHC rabbit polyclonal RO73 antibody and mAb 3F4 were used. Other immunohistochemical probes included rabbit polyclonal anti-bovine glial fibrillary acidic protein GFAP antibody (DAKO Corp), mAb anti-human Ab peptide (DAKO Corp.), polyclonal rabbit anti-human tau antibody (DAKO Corp.), and anti-phosphorylated high and middle molecular mass neurofilament protein (NFP) (Sternberger Monoclonals). The Bielschowsky silver stain was also used to search for neuritic plaques and neurofibrillary tangles.