Genetic variability of the prion protein gene (PRNP) in wild ruminants from Italy and Scotland [PDF]
The genetics of the prion protein gene (PRNP) play a crucial role in determining the relative susceptibility to transmissible spongiform encephalopathies (TSEs) in several mammalian species.
Acutis, Pier Luigi +33 more
core +1 more source
Is prnt a pseudogene? Identification of ram Prt in testis and ejaculated spermatozoa.
A hallmark of prion diseases or transmissible spongiform encephalopaties is the conversion of the cellular prion protein (PrP(C)), expressed by the prion gene (prnp), into an abnormally folded isoform (PrP(Sc)) with amyloid-like features that causes ...
Jorge Pimenta +15 more
doaj +1 more source
Gene expression profiling en association with prion-related lesions in the medulla oblongata of symptomatic natural scrapie animals. [PDF]
The pathogenesis of natural scrapie and other prion diseases remains unclear. Examining transcriptome variations in infected versus control animals may highlight new genes potentially involved in some of the molecular mechanisms of prion-induced ...
Bossers, A. +34 more
core +2 more sources
Gene-Edited Cell Models to Study Chronic Wasting Disease
Prion diseases are fatal infectious neurodegenerative disorders affecting both humans and animals. They are caused by the misfolded isoform of the cellular prion protein (PrPC), PrPSc, and currently no options exist to prevent or cure prion diseases ...
Simrika Thapa +5 more
doaj +1 more source
Proteinase K-Resistant Material in ARR/VRQ Sheep Brain Affected with Classical Scrapie Is Composed Mainly of VRQ Prion Protein [PDF]
Classical scrapie is a prion disease in sheep and goats. In sheep, susceptibility to disease is genetically influenced by single amino acid substitutions.
Bossers, A. +28 more
core +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 ...
Valerie Daggett +3 more
core +1 more source
Background Prion diseases are zoonotic diseases with a broad infection spectrum among mammalian hosts and are caused by the misfolded prion protein (PrPSc) derived from the normal prion protein (PrPC), which encodes the prion protein gene (PRNP ...
Yong-Chan Kim +2 more
doaj +1 more source
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 ...
Valerie Daggett +3 more
core +1 more source
Evolutionary descent of prion genes from the ZIP family of metal ion transporters. [PDF]
In the more than twenty years since its discovery, both the phylogenetic origin and cellular function of the prion protein (PrP) have remained enigmatic.
Gerold Schmitt-Ulms +4 more
doaj +1 more source
A novel protective prion protein variant that colocalizes with kuru exposure. [PDF]
BACKGROUND: Kuru is a devastating epidemic prion disease that affected a highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly affected adult women and children of both sexes.
Whittaker, John +26 more
core +1 more source

