Prion Protein Biology
نویسندگان
چکیده
منابع مشابه
Prion 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 کاملPrion protein: From physiology to cancer biology.
Prion protein (PrPc) was originally viewed solely as being involved in prion disease, but now several intriguing lines of evidence have emerged indicating that it plays a fundamental role not only in the nervous system, but also throughout the human body. PrPc is expressed most abundantly in the brain, but has also been detected in other non-neuronal tissues as diverse as lymphoid cells, lung, ...
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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...
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PrP (prion-related protein) is a cell-surface Cu(2+)-binding glycoprotein which, when misfolded, is responsible for a number of transmissible spongiform encephalopathies. The co-ordination geometry, stoichiometry and affinity of Cu(2+) for PrP are the subject of much debate. In the present paper, we review the recent progress we have made in these areas. As many as six Cu(2+) ions bind to PrP w...
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The conformational conversion of the cellular prion protein, PrP(C), to the misfolded isoform PrP(Sc )is the central pathogenic event in the uniquely transmissible neurodegenerative prion diseases. As both PrP(C) and PrP(Sc) are associated with membranes, the nature of the membrane microenvironment may well play a significant role in both the conformational conversion process as well as the nor...
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ژورنال
عنوان ژورنال: Cell
سال: 1998
ISSN: 0092-8674
DOI: 10.1016/s0092-8674(00)81163-0