منابع مشابه
Molecular biology of prion diseases.
Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann-Sträussler-Scheinker diseases of humans. Infectious prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein, which is encoded by a chromosomal gene. A posttranslational process, as yet ...
متن کاملCellular biology of prion diseases.
Prion diseases are fatal neurodegenerative disorders of humans and animals that are important because of their impact on public health and because they exemplify a novel mechanism of infectivity and biological information transfer. These diseases are caused by conformational conversion of a normal host glycoprotein (PrPC) into an infectious isoform (PrPSc) that is devoid of nucleic acid. This r...
متن کاملMolecular Pathogenesis of Prion Diseases
In humans, prion diseases occur with unique aetiology as sporadic, genetic or infectious disorders. Sporadic cases of prion diseases, which account for the majority of casualties (up to 85% of all cases), are of unknown origin; the genetic forms are less frequent (up to 15%), while the infectious cases are extremely rare with an incidence of less than 1% (Prusiner, 2001). Creutzfeldt-Jakob dise...
متن کاملMolecular basis of prion diseases.
Fatal neurodegenerative diseases have been known for more than 300 years. As early as 1759, the scrapie disease of sheep was mentioned as a lethal and transmissible disease in a german veterinary textbook by Leopoldt (1759). Experimental transmission was shown in 1936 by Cuillé (1938). Not knowing about the similarity to the sheep disease, the neurologists Alfons Jakob (1921) and Hans Creutzfel...
متن کاملThe molecular pathology of prion diseases.
15 Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal cellular prion protein (PrP) into a pathogenic likeness of itself (PrP). Formation of neurotoxic PrP and/or loss of the normal function o...
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ژورنال
عنوان ژورنال: Uirusu
سال: 1999
ISSN: 0042-6857,1884-3433
DOI: 10.2222/jsv.49.193