Prion protein
A receptor for infectious and cellular prion protein
... of protein and involved in transmissible spongiform encephalopathies in humans and ...anchored protein, PrPc, of unknown ...cellular prion protein causing conversion of the latters ...third ...
7
Distinct transmissibility features of TSE sources derived from ruminant prion diseases by the oral route in a transgenic mouse model (TgOvPrP4) overexpressing the ovine prion protein.
... murine prnp-/- background under the control of the neuron- specific enolase promoter [15]. This extends our previous demonstrations of the transmission of two classical scrapie isolates by intra-peritoneal route in this ...
8
Identification of single nucleotide polymorphisms in the prion protein gene in Santa Ines and Dorset sheep
... In this study, we analyzed the genotypes of Santa Ines and Dorset sheep in Brazil to determine their risk for scrapie, a fatal disease caused by a prion protein. And polymorphisms considered “rare” were ...
5
Prion protein expression regulates embryonic stem cell pluripotency and differentiation.
... Cellular prion protein (PRNP) is a glycoprotein involved in the pathogenesis of transmissible spongiform encephalopathies ...in prion infection has been extensively ...
12
The cellular prion protein negatively regulates phagocytosis and cytokine expression in murine bone marrow-derived macrophages.
... cellular prion protein (PrP C ) is a glycosylphosphatidylinositol (GPI)-anchored glycoprotein on the cell ...membrane protein-2 to phagosomes, suggesting enhanced microbicidal ...
9
Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
... Our findings raise the question of whether prions present in specific components of the skin such as peripheral nerve fibres may be involved in the natural transmission of scrapie. Dissemination of infectivity from the skin ...
9
Identification of the prion protein Doppel in spermatozoa and its impact in fertility
... (prion protein gene) is responsible for the expression of the cellular prion protein (PrP C ...downstream prion like protein, also known as Doppel or Dpl, which is not involved ...
72
Cellular prion protein expression is not regulated by the Alzheimer's amyloid precursor protein intracellular domain.
... and prion diseases fall within the spectrum of neurodegenerative diseases which are causally linked to misfolded and aggregated ...and prion diseases, and the proteins at the centre of these diseases; ...
8
Membrane-enriched proteome changes and prion protein expression during neural differentiation and in neuroblastoma cells
... the prion protein in the first cell model used in this study, (ii) interactors with PrP identified in our study are annotated mostly as “myelin sheat” associated proteins and therefore may mediate a ...
13
Rapid and Highly Sensitive Detection of Variant Creutzfeldt-Jakob Disease Abnormal Prion Protein on Steel Surfaces by Protein Misfolding Cyclic Amplification: Application to Prion Decontamination Studies.
... with prion-contaminated steel wires as the source of seeding infectious ...abnormal prion protein in 10 8 -fold dilutions of infected brain homoge- nate from either strain on a single wire, revealing ...
15
Intraepithelial and interstitial deposition of pathological prion protein in kidneys of scrapie-affected sheep.
... solely using immunohistochemistry Siso´ et al., [30] showed the presence of pathological prion protein, which they called PrP d . The authors found PrP d only in the renal medulla of scrapie sick sheep. By ...
8
Unexpected tolerance of alpha-cleavage of the prion protein to sequence variations.
... The cellular form of the prion protein, PrP C , undergoes extensive proteolysis at the a site (109KQH110). Expression of non- cleavable PrP C mutants in transgenic mice correlates with neurotoxicity, ...
11
Antimicrobial activity of human prion protein is mediated by its N-terminal region.
... Pep-screen peptides were from Sigma-Genosys, generated by a peptide synthesis platform (PEPscreen, Custom Peptide Libraries, Sigma Genosys) and yield was ,1–6 mg with an average crude purity of 60–70%. Prior to ...
11
Dopamine induces the accumulation of insoluble prion protein and affects autophagic flux
... of protein aggregates is a histopathological hallmark of several neurodegenerative diseases, but in most cases the aggregation occurs without defined mutations or clinical histories, suggesting that certain ...
11
Detection of prion protein particles in blood plasma of scrapie infected sheep.
... the prion consisting mainly, if not solely, of a misfolded and aggregated isoform of the host-encoded prion protein ...components. Prion protein aggregates were subsequently ...
8
The POM monoclonals: a comprehensive set of antibodies to non-overlapping prion protein epitopes.
... To confirm and explore the grouping of C-terminal POMs that resulted from pair-wise mapping data and to gain further insight on their binding specificities and cross-species reactivity, a panel of wild type and ...
17
Live-cell FRET imaging reveals clustering of the prion protein at the cell surface induced by infectious prions
... for prion conversion ...fluorescent protein (CFP and YFP, respectively) pair has been commonly used to measure FRET in cells due to their excitation and emission properties, suitable Förster distance and ...
11
The prion protein and New World primate phylogeny
... published prion protein DNA sequences for the following species (obtained from the genebank): Cercopithecus aethiops (U08291), Cercopithecus dianae (U08292), Aotus trivirgatus (U08293), Papio hamadryas ...
6
Prion protein (PrP) knock-out mice show altered iron metabolism: a functional role for PrP in iron uptake and transport.
... Prion protein (PrP) is a ubiquitously expressed cell surface glycoprotein linked to the plasma membrane by a glycosylpho- sphatidylinositol (GPI) ...human prion disorders, relatively less is known ...
14
Ablation of Prion Protein in Wild Type Human Amyloid Precursor Protein (APP) Transgenic Mice Does Not Alter The Proteolysis of APP, Levels of Amyloid-β or Pathologic Phenotype.
... of Prion Protein in Wild Type Human Amyloid Precursor Protein (APP) Transgenic Mice Does Not Alter The Proteolysis of APP, Levels of Amyloid-β or Pathologic ...
10