NR ASLY
AU Chesebro,B.; Trifilo,M.; Race,R.E.; Meade-White,K.; Teng,C.; LaCasse,R.; Raymond,L.D.; Favara,C.; Baron,G.S.; Priola,S.A.; Caughey,B.W.; Masliah,E.; Oldstone,M.
TI Anchorless prion protein results in infectious amyloid disease without clinical scrapie
QU Science 2005 Jun 3; 308(5727): 1435-9
KI Science. 2005 Jun 3;308(5727):1420-1. PMID: 15933188
PT journal article
AB In prion and Alzheimer's diseases, the roles played by amyloid versus nonamyloid deposits in brain damage remain unresolved. In scrapie-infected transgenic mice expressing prion protein (PrP) lacking the glycosylphosphatidylinositol (GPI) membrane anchor, abnormal protease-resistant PrPres was deposited as amyloid plaques, rather than the usual nonamyloid form of PrPres. Although PrPres amyloid plaques induced brain damage reminiscent of Alzheimer's disease, clinical manifestations were minimal. In contrast, combined expression of anchorless and wild-type PrP produced accelerated clinical scrapie. Thus, the PrP GPI anchor may play a role in the pathogenesis of prion diseases.
MH Animals; Brain/metabolism/pathology/ultrastructure; Glycosylphosphatidylinositols/genetics/*metabolism; Mice; Mice, Inbred C57BL; Mice, Transgenic; PrPsc Proteins/chemistry/metabolism; Prion Diseases/etiology/metabolism/pathology; Prions/biosynthesis/chemistry/genetics/*metabolism; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, P.H.S.; Scrapie/*etiology/metabolism/pathology; Senile Plaques/metabolism/pathology
AD Bruce Chesebro (bchesebro@niaid.nih.gov), Richard Race, Kimberly Meade-White, Rachel LaCasse, Lynne Raymond, Cynthia Favara, Gerald Baron, Suzette Priola, Byron Caughey, Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Hamilton, MT 59840, USA; Matthew Trifilo, Chao Teng, Michael Oldstone, Division of Virology, Department of Neuropharmacology, Scripps Research Institute, La Jolla, CA 92037, USA; Eliezer Masliah, Departments of Neurosciences and Pathology, University of California, San Diego, La Jolla, CA 92093, USA
SP englisch
PO USA