Molecular biology of prion diseases

SB Prusiner - Science, 1991 - science.org
Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and
bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann …

A'unified theory'of prion propagation

C Weissmann - Nature, 1991 - nature.com
There is now very persuasive evidence that the transmissible agent for spongiform
encephalopathies such as scrapie, consists of a modified form of the normal host protein …

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication

SB Prusiner, M Scott, D Foster, KM Pan, D Groth… - Cell, 1990 - cell.com
Transgenic (Tg) mice expressing both Syrian hamster (Ha) and mouse (Mo) prion protein
(PrP) genes were used to probe the mechanism of scrapie prion replication. Four Tg lines …

N-terminal truncation of the scrapie-associated form of PrP by lysosomal protease (s): implications regarding the site of conversion of PrP to the protease-resistant …

B Caughey, GJ Raymond, D Ernst, RE Race - Journal of virology, 1991 - Am Soc Microbiol
Scrapie and related transmissible spongiform encephalopathies result in the accumulation
of a protease-resistant form of an endogenous brain protein called PrP. As an approach to …

Prion protein biosynthesis in scrapie-infected and uninfected neuroblastoma cells

B Caughey, RE Race, D Ernst, MJ Buchmeier… - Journal of …, 1989 - Am Soc Microbiol
Numerous studies have indicated that a modified proteinase K-resistant form of an
endogenous brain protein, prion protein (PrP), is associated with scrapie infection in …

Characterization of scrapie infection in mouse neuroblastoma cells

RE Race, LH Fadness… - Journal of General …, 1987 - microbiologyresearch.org
A mouse neuroblastoma cell line was successfully infected with scrapie agent. Agent
derived from infected mouse brain or spleen infected cultures. However, agent from infected …

Immunoaffinity purification and neutralization of scrapie prion infectivity.

R Gabizon, MP McKinley, D Groth… - Proceedings of the …, 1988 - National Acad Sciences
Prions are unusual infectious pathogens causing scrapie of sheep and goats as well as
Creutzfeldt-Jakob disease of humans. Biochemical and genetic studies contend that the …

Biology and genetics of prion diseases

SB Prusiner - Annual review of microbiology, 1994 - go.gale.com
Prions are infectious proteins that have been implicated in various human diseases such as
Gertsmann-Straussler-Scheinker disease, Creutzfeldt-Jacob disease and Kuru disease. The …

Human prion protein cDNA: molecular cloning, chromosomal map**, and biological implications

YC Liao, RV Lebo, GA Clawson, EA Smuckler - Science, 1986 - science.org
A human complementary DNA whose protein product is considered to be the major
component of scrapie-associated fibrils in Creutzfeldt-Jakob disease, kuru, and Gerstmann …

Genetic and infectious prion diseases

SB Prusiner - Archives of neurology, 1993 - jamanetwork.com
Enriching fractions from Syrian hamster (SHa) brain for scrapie prion infectivity led to the
discovery of the prion protein (PrP). Prion diseases include scrapie of sheep and bovine …