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 کاملMolecular biology of prion protein and its first homologous protein.
Conformational conversion of the normal cellular isoform of prion protein, PrP(C), a glycoprotein anchored to the cell membrane by a glycosylphosphatidylinositol moiety, into the abnormally folded, amyloidogenic prion protein, PrP(Sc), plays a pivotal role in the pathogenesis of prion diseases. It has been suggested that PrP(C) might be functionally disturbed by constitutive conversion to PrP(S...
متن کاملPrion Protein Biology
and seem to be composed exclusively of a modified Stanley B. Prusiner,*† Michael R. Scott,* Stephen J. DeArmond,‡* and Fred E. Cohen†§‖ isoform of PrP, designated PrP. The normal cellular PrP, denoted PrP, is converted into PrP through a *Department of Neurology †Department of Biochemistry and Biophysics process whereby a portion of its a-helical and coil structure is refolded into b sheet (Pan...
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...
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ژورنال
عنوان ژورنال: The journal of animal genetics
سال: 2006
ISSN: 1884-3883,1344-9265
DOI: 10.5924/abgri2000.34.2_71