Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type
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چکیده
منابع مشابه
Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type
In most human sporadic prion diseases the phenotype is consistently associated with specific pairings of the genotype at codon 129 of the prion protein gene and conformational properties of the scrapie PrP (PrPSc) grossly identified types 1 and 2. This association suggests that the 129 genotype favours the selection of a distinct strain that in turn determines the phenotype. However, this mecha...
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Introduction. Genetic prion diseases, such as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), Fatal Familial Insomnia (FFI), are associated with the mutations of prion (PRNP) gene. More than 30 pathogenic PRNP mutations were identified. Since Alzheimer disease (AD) and prion diseases have many similarities in the neuropathological and physiological symptoms, we te...
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Prion diseases are a group of infectious fatal neurodegenerative diseases. The conformational conversion of a cellular prion protein (PrP(C)) into an abnormal misfolded isoform (PrP(Sc)) is the key event in prion diseases pathology. Under normal conditions, the high-energy barrier separates PrP(C) from PrP(Sc) isoform. However, pathogenic mutations, modifications as well as some cofactors, such...
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Prion diseases, or transmissible spongiform encephalopathies (TSEs) are progressive, fatal neurodegenerative diseases with no effective treatment. The pathology of these diseases involves the conversion of a protease sensitive form of the cellular prion protein (PrP(C)) into a protease resistant infectious form (PrP(res)). The efficiency of this conversion is predicated upon a number of factors...
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ژورنال
عنوان ژورنال: Journal of Neurochemistry
سال: 2005
ISSN: 0022-3042,1471-4159
DOI: 10.1111/j.1471-4159.2004.02859.x