Subclinical scrapie infection in a resistant species: persistence, replication, and adaptation of infectivity during four passages.

Long-term subclinical carrier state precedes scrapie replication and adaptation in a resistant species: analogies to bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease in humans.

Cattle infected with bovine spongiform encephalopathy (BSE) appear to be a reservoir for transmission of variant Creutzfeldt-Jakob disease (vCJD) to humans. Although just over 100 people have developed clinical vCJD, millions have probably been exposed to the infectivity by consumption of BSE-infected beef. It is currently not known whether some of these individuals will develop disease themsel...

Long-Term Subclinical Carrier S tate Precedes Scrapie Replication and Adaptation in a Resistant Species: Analogies to Bovine Spongiform Enckphalopathy and Variant Creutzfeldt-Jakob Disease in Humans

The Role of GPI-anchored PrPC 119 The Role of GPI-anchored PrP in Mediating the Neurotoxic Effect of Scrapie Prions in Neurons

There are two central phenomena in prion disease: prion replication and prion neurotoxicity. Underlying them both is the conversion of a host-encoded ubiquitously expressed protein, prion protein (PrPC), into a partially-protease resistant isoform, PrPSc, which accumulates in the brain. PrPSc is associated with both pathology and infectivity (Prusiner, 1991). In the absence of PrPC, PrPSc canno...

Intrinsic resistance of oligodendrocytes to prion infection.

Within the CNS, the normal form of cellular prion protein (PrP(C)) is expressed on neurons, oligodendrocytes, and astrocytes. The contribution of these cell types to prion replication and pathogenesis is unclear. To assess the role of oligodendrocytes, we expressed PrP(C) under the control of the myelin basic protein (MBP) promoter in mice lacking endogenous PrP(C). PrP(C) was detected in oligo...

Congo red inhibition of scrapie agent replication.

Congo red inhibits the accumulation of protease-resistant PrP in scrapie-infected mouse neuroblastoma cells. Here we show that Congo red also inhibits the replication of scrapie infectivity in these cells. This observation is consistent with the idea that protease-resistant PrP is a vital component of the scrapie agent or that agent replication depends on the presence of protease-resistant PrP ...