NR ARJG
AU Politopoulou,G.
TI Experimental approaches to TSE prevention via inhibition of prion formation
QU Protein and Peptide Letters 2004 Jun; 11(3): 249-55
PT journal article; review; review, tutorial
AB Transmissible spongiform encepahalopathies (TSEs) are fatal diseases that damage the central nervous system. TSEs are unique in that they may be inherited, infectious or spontaneous. The central pathogenic agent is thought to be a conformationally distinct form (PrP(Sc;)) of the endogenous prion protein(PrPc), which is high in beta-sheet content and is resistant to proteases; infectivity is thought to involve formation of PrPsc via imprinting of abnormal conformation on the normal form of the protein (PrPc) by seeds of PrPsc. A number of compounds found to inhibit the conversion of PrPc to PrPsc have been proposed as therapeutics to halt TSEs.
ZR 38
MH Animals; Humans; Peptides/chemistry/pharmacology/therapeutic use; Prion Diseases/*metabolism/*prevention & control/therapy; Prions/*antagonists & inhibitors/chemistry/*metabolism/pathogenicity
AD Department of Rheumatology, Avon Orthopaedic Centre, Southmead Hospital, University of Bristol. g.politopoulou@bristol.ac.uk
SP englisch
PO Niederlande