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Prion Pathway

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Prion diseases or TSEs (Transmissible Spongiform Encephalopathies) form a biologically unique group of infectious fatal neurodegenerative disorders, which are caused by toxic gain of function in a normal host cell protein (the Prion protein, PrP). The mechanism of disease propagation is well understood and involves the conformational conversion of a normal cell-surface protein (PrPc) into a protease-resistant, Beta-sheet-rich form (PrPSc) that is infectious in the absence of nucleic acid. BSE (Bovine Spongiform Encephalopathy), Scrapie of sheep and goat, CWD (Chronic Wasting Disease) of deer and elk, and human CJD (Creutzfeldt Jakob Disease) are well-known central nervous system degenerative diseases caused by Prion infection. However, some forms of spongiform encephalopathies are commonly associated with inherited mutations at the PrPc coding gene [...]


1.Biochemistry and structure of PrP(C) and PrP(Sc).
Riesner D.
Br Med Bull. 2003;66:21-33.
2.Prion protein trafficking and the development of neurodegeneration.
Hegde RS, Rane NS.
Trends Neurosci. 2003 Jul;26(7):337-9.
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