The role of the prion protein membrane anchor in prion infection.
Normal cellular and abnormal disease-associated forms of prion protein (PrP) contain a C-terminal glycophosphatidyl-inositol (GPI) membrane anchor. The importance of the GPI membrane anchor in prion diseases is unclear but there are data to suggest that it both is and is not required for abnormal prion protein formation and prion infection. Utilizing an in vitro model of prion infection we have recently demonstrated that, while the GPI anchor is not essential for the formation of abnormal prion protein in a cell, it is necessary for the establishment of persistent prion infection. In combination with previously published data, our results suggest that GPI anchored PrP is important in the amplification and spread of prion infectivity from cell to cell.
Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Hamilton, MT, USA. spriola@niaid.nih.gov
Suzette A
SA
Priola
Kristin L
KL
McNally
D000818:Animals; D001921:Brain / Q000473:pathology; D004195:Disease Models, Animal; D000939:Epitopes / Q000737:chemistry; D017261:Glycosylphosphatidylinositols / Q000737:chemistry; D006801:Humans; D007425:Intracellular Membranes / Q000378:metabolism*; D051379:Mice; D017096:Prion Diseases / Q000378:metabolism / Q000503:physiopathology*; D011328:Prions / Q000378:metabolism*; D012608:Scrapie / Q000503:physiopathology; D013997:Time Factors
D000939:Epitopes; D017261:Glycosylphosphatidylinositols; D011328:Prions