... to harbour different pathogenic potential for host cells. Most significantly, α-cleavage produces a C-terminal fragment that is resistant to folding into soluble β-strand-rich toxic isoforms. α-cleavage neurotoxicity peptide modelling prion protein (PrP) prion protein cleavage Fresh...

... neurodegenerative diseases linked to mutations in the PRNP gene encoding the cellular prion protein (PrP C ). How sequence variants of PRNP encode the information to specify these disease phenotypes is not known. It is suggested that each mutation produces a misfolded variant of PrP C with specific neurotoxic...

... disease prion protein (PrP) protein conformation transgenic Mammalian prion diseases are fatal neurodegenerative conditions of humans and various other vertebrate species. These diseases include scrapie in sheep, BSE (bovine spongiform encephalopathy) in cattle and CJD (Creutzfeldt–Jakob...

... be addressed to either of these authors (email [email protected] or [email protected] ). 20 4 2010 24 6 2010 14 7 2010 14 7 2010 © The Authors Journal compilation © 2010 Biochemical Society 2010 inherited prion disease prion protein (PrP) protein aggregation...

...Anna Caputo; Daniela Sarnataro; Vincenza Campana; Maddalena Costanzo; Alessandro Negro; M. Catia Sorgato; Chiara Zurzolo Dpl (doppel) is a paralogue of the PrP C (cellular prion protein), whose misfolded conformer (the scrapie prion protein, PrP Sc ) is responsible for the onset of TSEs...

... PNGase F peptide N-glycosidase F PrP prion protein PrP C cellular PrP PrP Sc pathogenic PrP RML Rocky Mountain Laboratory vCJD variant CJD This work was funded by the U.K. Medical Research Council. References 1 Prusiner S. B. Prions Proc. Natl...

... The Authors Journal compilation © 2008 Biochemical Society 2008 binding affinity co-ordination copper octapeptide prion protein (PrP) protein stability PrP (prion protein) is a cell-surface glycoprotein that has been directly implicated in the pathogenesis of a range of neurodegenerative...

...Mark Klewpatinond; John H. Viles The prion protein (PrP) is a Cu 2+ -binding cell-surface glycoprotein. Using various PrP fragments and spectroscopic techniques, we show that two Cu 2+ ions bind to a region between residues 90 and 126. This region incorporates the neurotoxic portion of PrP, vital...

..., Australia. 2 To whom correspondence should be addressed (email [email protected] ). 5 5 2006 17 8 2006 8 9 2006 8 9 2006 The Biochemical Society, London 2007 CD spectroscopy folding intermediate prion protein (PrP) secondary structure translational diffusion...

... ). 16 5 2006 20 7 2006 22 8 2006 22 8 2006 The Biochemical Society, London 2006 copper (II) equilibrium dialysis prion protein (PrP) self-association Prion diseases are a group of transmissible neurodegenerative disorders, found in both humans and animals, which...

... The Biochemical Society, London 2006 Copper (II) fluorescence metal binding NMR prion prion protein (PrP) Prion diseases are a group of fatal neurodegenerative disorders that includes Creutzfeldt–Jakob disease in humans, scrapie in sheep and bovine spongiform encephalopathy in cattle. In humans...

... CJD Creutzfeldt–Jacob disease PrP prion protein PrP C normal cellular PrP PrP Sc abnormal disease-specific conformation of PrP SOD superoxide dismutase V C background control rate of dismutase activity V S sample assay activity XO xanthine oxidase...

..., heated (95 °C, 5 min) and loaded on to the gels. cauda epididymal plasma clusterin epididymis glycosylphosphatidylinositol (GPI) anchor hydrophobic protein prion protein (PrP) PrP C (cellular PrP, where PrP stands for prion protein) is a glycoprotein of unknown function, highly...

...Henrik MÜLLER; Alexander STROM; Gerhard HUNSMANN; Andreas W. STUKE The conformational conversion of the normal cellular prion protein (PrP C ) into the pathology-associated PrP Sc isoform is a key event in TSEs (transmissible spongiform encephalopathies). The host PrP C molecule contains two N...

... of susceptibility and resistance to prion disease. 1 To whom correspondence should be addressed (e-mail [email protected] ). 18 11 2003 7 1 2004 18 2 2004 18 2 2004 The Biochemical Society, London ©2004 2004 conformational change α-helix copper metal ion prion protein (PrP...