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PMID: 7937921 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein.

Proceedings of the National Academy of Sciences of the United States of America ·Vol. 91 ·No. 21 ·1994-10-11 ·Pages 9936-40

Telling GC, Scott M, Hsiao KK, Foster D, Yang SL, Torchia M, Sidle KC, Collinge J, DeArmond SJ, Prusiner SB

Abstract

Transgenic (Tg) mice were constructed that express a chimeric prion protein (PrP) in which a segment of mouse (Mo) PrP was replaced with the corresponding human (Hu) PrP sequence. The chimeric PrP, designated MHu2MPrP, differs from MoPrP by 9 amino acids between residues 96 and 167. All of the Tg(MHu2M) mice developed neurologic disease approximately 200 days after inoculation with brain homogenates from three patients dying of Creutzfeldt-Jakob disease (CJD). Inoculation of Tg(MHu2M) mice with CJD prions produced MHu2MPrPSc (where PrPSc is the scrapie isoform of PrP); inoculation with Mo prions produced Mo-PrPSc. The patterns of MHu2MPrPSc and MoPrPSc accumulation in the brains of Tg(MHu2M) mice were different. About 10% of Tg(HuPrP) mice expressing HuPrP and non-Tg mice developed neurologic disease > 500 days after inoculation with CJD prions. The different susceptibilities of Tg(HuPrP) and Tg(MHu2M) mice to Hu prions indicate that additional species-specific factors are involved in prion replication. Diagnosis, prevention, and treatment of Hu prion diseases should be facilitated by Tg(MHu2M) mice.

MeSH Terms
Animals Astrocytes/metabolism,pathology Brain/metabolism,pathology Creutzfeldt-Jakob Syndrome/genetics,pathology,physiopathology Humans Mice Mice, Transgenic Open Reading Frames Polymerase Chain Reaction/methods Prions/analysis,biosynthesis,genetics Recombinant Fusion Proteins/analysis,biosynthesis Restriction Mapping Time Factors
Chemicals
Prions Recombinant Fusion Proteins
Authors & Affiliations
10 authors, click to expand affiliations / ORCID
Telling G C
Department of Neurology, University of California, San Francisco 94143.
Scott M
Hsiao K K
Foster D
Yang S L
Torchia M
Sidle K C
Collinge J
DeArmond S J
Prusiner S B
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Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
DOI
10.1073/pnas.91.21.9936
Published
1994-10-11
Pages
9936-40
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC44932
Subset
IM
Grants
Wellcome Trust · United Kingdom
External Links
PubMed source DOI: 10.1073/pnas.91.21.9936 PMC Full Text View journal details
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