منابع مشابه
Prion Diseases
Stanley Prusiner introduced the term prion (proteinaceous infectious particle) and proposed that the infectious agent in prion diseases is composed mainly or entirely of an abnormal conformation of an otherwise normal host-encoded glycoprotein called the prion protein (PrP)1. This hypothesis was initially greeted with great skepticism in the scientific community but then later was widely acknow...
متن کاملNeuropathology of prion diseases.
In prion diseases, neuropathology has remained the most important tool to give a definite diagnosis, and neuropathological research has contributed significantly to our current pathogenetic understanding. Immunohistochemistry for the disease-associated prion protein (PrP(Sc)) is indispensable for the neuropathological confirmation of prion diseases. The amount and distribution of PrP(Sc) deposi...
متن کاملPeculiarities of Prion Diseases
Prion diseases (PrDs) are transmissible and fatal neurodegenerative diseases naturally occurring in humans and animals, ‘‘mad cow’’ disease being the most infamous. Their development and propagation requires endogenous prion protein (PrP) and derives from the conversion of PrP to a misfolded form, which combines with other misfolded PrP molecules to form small nuclei (seeds). The seeds can then...
متن کاملPrion and Prion-like Diseases in Humans
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...
متن کاملCopper and prion diseases.
Transmissible spongiform encephalopathies are diseases of animals and humans that are also termed prion diseases. These diseases are linked together because a normal brain glycoprotein termed the prion protein is converted to a readily detectable protease-resistant isoform. There is now strong evidence to suggest that apart from this difference in resistance a major difference between the isofo...
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ژورنال
عنوان ژورنال: Journal of Magnetic Resonance Imaging
سال: 2012
ISSN: 1053-1807
DOI: 10.1002/jmri.23504