Tracking the elusive prion.
Prion diseases are infectious neurodegenerative fatal disorders. There are currently no treatments or cures. Considerable evidence suggests that the infectious agent is an abnormally folded protein that promotes or seeds its normal cellular isoform to fold into the infectious form. However, the precise mechanism and factors involved in this conversion remain unknown. A major stumbling block to further investigation has been the inability to seed the formation of new infectious material in vitro. Now, however, infectious material has been generated in a cell-free system. Although this system uses cell lysate rather than pure proteins, it nevertheless opens the door to the elucidation of targets of intervention and the development of useful diagnostic and therapeutic approaches.
Liebman
Susan W
SW
Department of Biological Sciences, The University of Illinois at Chicago, Chicago, IL 60670, USA. sueL@uic.edu
Mastrianni
James A
JA
D000818:Animals; D001921:Brain / Q000378:metabolism*; D002474:Cell-Free System; D003001:Cloning, Molecular / Q000379:methods*; D021141:Nucleic Acid Amplification Techniques / Q000379:methods*; D018620:PrPSc Proteins / Q000737:chemistry*; D017510:Protein Folding
D018620:PrPSc Proteins