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Trapping Prion Protein in the Endoplasmic Reticulum Impairs PrPC Maturation and Prevents PrPSc Accumulation*
The conversion of the normal cellular prion protein (PrPC) into the abnormal scrapie isoform (PrPSc) is a key feature of prion diseases. The pathogenic mechanisms and the subcellular sites of theExpand
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Regulation of intrinsic prion protein by growth factors and TNF-alpha: the role of intracellular reactive oxygen species.
Function and regulation of the intrinsic prion protein (PrPc) are largely unknown. In the present study the regulation of PrPc expression by growth factors and cytokines that increase intracellularExpand
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Scrapie infectivity is quickly cleared in tissues of orally-infected farmed fish
BackgroundScrapie and bovine spongiform encephalopathy (BSE) belongs to the group of animal transmissible spongiform encephalopathy (TSE). BSE epidemic in the UK and elsewhere in Europe has beenExpand
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Molecular diagnostics of transmissible spongiform encephalopathies.
Clinical criteria for the diagnosis of sporadic, iatrogenic and variant Creutzfeldt-Jakob diseases are now available and show an excellent sensitivity and specificity ( approximately 98%).Expand
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Expression of wild-type and V210I mutant prion protein in human neuroblastoma cells
The conversion of the host-encoded prion protein (PrPc) into the insoluble, protease-resistant isoform (PrPsc) is the main pathogenic mechanism of transmissible spongiform encephalopathies. They areExpand
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KDEL-tagged anti-prion intrabodies impair PrP lysosomal degradation and inhibit scrapie infectivity.
Transmissible spongiform encephalopathy or prion diseases are fatal neurodegenerative disorders characterized by the conversion of the cellular prion protein (PrPC) into the infectious scrapieExpand
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Oral pravastatin prolongs survival time of scrapie-infected mice.
Statins are potent inhibitors of HMG-CoA (3-hydroxy-3-methylglutaryl coenzyme A) reductase in the cholesterol-biosynthesis pathway. They are either lipophilic (e.g. simvastatin) or hydrophilic [e.g.Expand
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Accumulation and aberrant composition of cholesteryl esters in Scrapie-infected N2a cells and C57BL/6 mouse brains
ObjectiveCholesterol changes have been described in prion-cell models and in experimental rodent scrapie; yet, the pattern of this association is still controversial.MethodsTo shed light on theExpand
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Safety of milk and milk derivatives in relation to BSE: the lactoferrin example
Bovine Spongiform Encephalopathy (BSE) belongs to Transmissible Spongiform Encephalopathies (TSEs) or Prion diseases. BSE is a feed borne infection of cattle. Epidemiological and laboratory dataExpand
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