BackgroundExisting mathematical models for scrapie dynamics in sheep populations assume that the PrP gene is only associated with scrapie susceptibility and with no other fitness related traits.
Andrea Doeschl-Wilson +3 more
doaj +1 more source
Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]
Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
core +2 more sources
Evidence in sheep for pre-natal transmission of scrapie to lambs from infected mothers.
Natural scrapie transmission from infected ewes to their lambs is thought to occur by the oral route around the time of birth. However the hypothesis that scrapie transmission can also occur before birth (in utero) is not currently favoured by most ...
James D Foster +2 more
doaj +1 more source
Background Susceptibility of sheep to scrapie infection is known to be modulated by the PrP genotype of the animal. In the Netherlands an ambitious scrapie control programme was started in 1998, based on genetic selection of animals for breeding.
Engel Bas +5 more
doaj +1 more source
Anti-prion drug mPPIg5 inhibits PrP(C) conversion to PrP(Sc). [PDF]
Prion diseases, also known as transmissible spongiform encephalopathies, are a group of fatal neurodegenerative diseases that include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans.
A Ertmer +49 more
core +3 more sources
A novel, resistance-linked ovine PrP variant and its equivalent mouse variant modulate the in vitro cell-free conversion of rPrP to PrPres [PDF]
Prion diseases are associated with the conversion of the normal cellular prion protein, PrPc, to the abnormal, disease-associated form, PrPSc. This conversion can be mimicked in vitro by using a cell-free conversion assay. It has recently been shown that
Gill, Andrew C. +4 more
core +1 more source
Effect of incineration, co-incineration and combustion on TSE hazards in category 1 animal by-products. [PDF]
Abstract The European Commission requested EFSA to assess the effect of incineration, co‐incineration and combustion of Category 1 animal by‐products (ABP) on the BSE/TSE hazards in ash resulting from these treatments. The presence of residual TSE hazards is assessed by detection of prion infectivity or seeding activity.
EFSA Panel on Biological Hazards (BIOHAZ) +24 more
europepmc +2 more sources
Molecular dynamics as an approach to study prion protein misfolding and the effect of pathogenic mutations [PDF]
Computer simulation of protein dynamics offers unique high-resolution information that complements experiment. Using experimentally derived structures of the natively folded prion protein (PrP), physically realistic dynamics and conformational changes ...
Daggett, Valerie, van der Kamp, Marc W
core +2 more sources
Selection for scrapie resistance decreased inbreeding rates in two rare sheep breeds in the Netherlands [PDF]
An attempt to balance selection intensity and conservation of genetic diversity was made in the national program for breeding for scrapie resistance in the Netherlands. The ARR allele is associated with resistance to scrapie.
Hiemstra, S.J. +2 more
core +16 more sources
Transmissibility of atypical scrapie in ovine transgenic mice: major effects of host prion protein expression and donor prion genotype. [PDF]
Atypical scrapie or Nor98 has been identified as a transmissible spongiform encephalopathy (TSE) that is clearly distinguishable from classical scrapie and BSE, notably regarding the biochemical features of the protease-resistant prion protein PrP(res ...
Jean-Noël Arsac +7 more
doaj +1 more source

