... induces accumulations of an abnormal form of prion protein (PrPres) in nervous and lymphoid tissues. This report ... ...
NBII National Biological Information Infrastructure
Prion diseases are fatal neurodegenerative diseases resulting from misfolding of normal cellular prion (PrP**C) into an abnormal form of scrapie prion (PrP**Sc). The cellular mechanisms underlying the misfolding of PrP**C are not well understood. Since cellular prion ...
Technology Transfer Automated Retrieval System (TEKTRAN)
The transmissible spongiform encephalopathies of domestic livestock are fatal neurodegenerative disorders characterized by accumulation of an abnormal isoform of the host prion protein in brain. Scrapie is the prion disease of sheep and the abnormal protein usually ...
... abstract (free full-text available): Analysis of abnormal prion protein glycoform patterns from chronic wasting disease (CWD)- ... ...
... encephalopathies (TSEs) are thought to be caused by the accumulation of abnormal protease-resistant proteins called prions, which are found in ...
DTIC Science & Technology
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurological disorder characterized by abnormal deposits of a protease-resistant isoform of the prion protein. Characterizing linkage disequilibrium (LD) and haplotype networks within the bovine prion gene (PRNP) is important for 1) test...
... Both diseases are transmissible spongiform encephalopathies (TSE), or prion diseases, caused by autocatalytic conversion of endogenously encoded prion protein (PrP) to an abnormal, neurotoxic conformation ...
Prions typically accumulate in nervous and lymphoid tissues. Because proinflammatory cytokines and immune cells are required for lymphoid prion replication, we tested whether inflammatory conditions affect prion pathogenesis. We administered prions to mice with five inflammatory diseases of the kidney, pancreas, or ...
NASA Astrophysics Data System (ADS)
Prion is an infectious particle composed of an abnormal isoform of the prion protein (PrPSc) and causes prion diseases such as bovine spongiform encephalopathy (BSE), Creutzfeldt-Jakob disease (CJD) and scrapie. Host cells express cellular prion protein ...
PubMed
Prion diseases are characterized by the presence of the abnormal scrapie isoform of prion protein (Prp(exp Sc)) in affected brains. A conformational change is believed to convert the normal cellular prion protein (PrP (expC)) into PrP(exp Sc). Detection o...
National Technical Information Service (NTIS)
... lacking the glycophosphatydylinositol membrane anchor. In the brain, blood, and heart, both abnormal protease-resistant prion protein ( ... nontransgenic recipients. The titer of infectious scrapie in bloo...
Bovine spongiform encephalopathy (BSE) is a fatal neurological disorder characterized by abnormal deposits of a protease-resistant isoform of the prion protein. In previous studies, we have characterized linkage disequilibrium (LD) and haplotype networks within the bovine prion gene (PRNP). Other ...
... only): A captive adult male white-tailed deer (Odocoileus virginianus) with wasting and neurologic signs similar to chronic ... ...
... it possible to detect the abnormal protein in blood. A peptide from the carboxyl terminal region, amino ... labeled peptide in the capillary was 50 amol. Blood was obtained from normal sheep and elk, from...
The key event in the pathogenesis of prion diseases is the conversion of normal cellular prion proteins (PrP**c) to the proteinase K (PK) resistant, abnormal form (PrP**sc); however, the cellular mechanisms underlying the conversion remain enigmatic. Binding of divalent cations such as copper to th...
Background: The key biochemical event in the pathogenesis of prion diseases is the conversion of normal cellular prion proteins (PrP**c) to the proteinase K (PK) resistant, abnormal form (PrP**sc); however, the cellular mechanisms underlying the conversion remain enigmatic. Binding of divalent ca...
The transmissible spongiform encephalopathies are a family of fatal neurodegenerative diseases characterized by accumulation of abnormal prion proteins in the brain. The abnormal prion protein is the major constituent of the infectious agent and is a reliable marker for ...
Bovine spongiform encephalopathy (BSE) is a fatal neurological disorder characterized by abnormal deposits of a protease-resistant isoform of the prion protein. Typical and atypical BSEs have been identified in cattle and the relationships of prion gene (PRNP) variation with susceptibility to these...
