Abstract:
:To investigate the role of the pathogenic prion protein (PrP(Sc)) in controlling susceptibility to foreign prions, two Syrian hamster (SHa) prion strains, Sc237 and DY, were transmitted to transgenic mice expressing chimeric SHa/mouse PrP genes, Tg(MH2M). First passage of SHa(Sc237) prions exhibited prolonged incubation times, diagnostic of a species barrier. PrP(Sc) of the new MH2M(Sc237) strain possessed different structural properties from those of SHa(Sc237), as demonstrated by relative conformational stability measurements. This change was accompanied by a disease phenotype different from the SHa(Sc237) strain. Conversely, transmission of SHa(DY) prions to Tg(MH2M) mice showed no species barrier, and the MH2M(DY) strain retained the conformational and disease-specific properties of SHa(DY). These results suggest a causal relationship between species barriers, changes in PrP(Sc) conformation, and the emergence of new prion strains.
journal_name
Neuronjournal_title
Neuronauthors
Peretz D,Williamson RA,Legname G,Matsunaga Y,Vergara J,Burton DR,DeArmond SJ,Prusiner SB,Scott MRdoi
10.1016/s0896-6273(02)00726-2subject
Has Abstractpub_date
2002-06-13 00:00:00pages
921-32issue
6eissn
0896-6273issn
1097-4199pii
S0896627302007262journal_volume
34pub_type
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