In situ formation of protease-resistant prion protein in transmissible spongiform encephalopathy-infected brain slices.

The Journal of Biological Chemistry
R A BessenB Caughey

Abstract

The transmissible spongiform encephalopathies (TSEs) comprise a group of fatal neurodegenerative diseases that are characterized by the conversion of the normal host cellular prion protein (PrPC), to the abnormal protease-resistant prion protein isoform (PrP-res). It has been proposed, though not proven, that the infectious TSE agent consists solely of PrP-res and that PrP-res-induced conformational conversion of PrPC to additional PrP-res represents agent replication. In this study we demonstrate in situ conversion of protease-sensitive PrPC to PrP-res in TSE-infected brain slices. One step in this process is the binding of soluble PrPC to endogenous PrP-res deposits. The newly formed PrP-res associated with the slices in a pattern that correlated with the pre-existing brain distribution of PrP-res. Punctate in situ PrP conversion was observed in brain regions containing PrP-res amyloid plaques, and a more dispersed conversion product was detected in areas containing diffuse PrP-res deposits. These studies provide direct evidence that PrP-res formation involves the incorporation of soluble PrPC into both nonfibrillar and fibrillar PrP-res deposits in TSE-infected brain. Our findings suggest that the in situ PrP conversion reac...Continue Reading

References

Feb 13, 1992·The New England Journal of Medicine·R MedoriP Cortelli
Feb 1, 1992·The Journal of General Virology·R A Bessen, R F Marsh
Jun 15, 1992·Proceedings of the National Academy of Sciences of the United States of America·J E MaggioP W Mantyh
Jun 14, 1991·Science·S B Prusiner
Dec 9, 1991·FEBS Letters·J P Liautard
Dec 1, 1987·Journal of Virology·R J KascsakH Diringer
Jan 1, 1989·Acta Neuropathologica·A D SnowS J DeArmond
Jan 1, 1989·The Journal of General Virology·R A SomervilleP H Gibson
Apr 1, 1986·Proceedings of the National Academy of Sciences of the United States of America·R K MeyerS B Prusiner
Aug 1, 1986·Annals of Neurology·T KitamotoS B Prusiner
Oct 31, 1987·The Veterinary Record·G A WellsR Bradley
Sep 2, 1967·Nature·J S Griffith
Dec 1, 1983·Cell·S B PrusinerG G Glenner
Dec 1, 1983·Nature·H DiringerR H Kimberlin
Jan 1, 1981·Acta Neuropathologica·P A MerzK Iqbal
Apr 25, 1995·Proceedings of the National Academy of Sciences of the United States of America·D A KociskoB Caughey
Dec 1, 1993·Proceedings of the National Academy of Sciences of the United States of America·K M PanF E Cohen
Aug 11, 1994·Nature·D A KociskoB Caughey
Sep 9, 1994·Science·P T Lansbury
Sep 9, 1994·Science·P R Epstein, G P Chikwenhere
Jul 1, 1993·Proceedings of the National Academy of Sciences of the United States of America·J H ComeP T Lansbury
Apr 6, 1996·Lancet·R G WillP G Smith
Jul 1, 1996·Journal of Virology·F EdenhoferE L Winnacker
Dec 1, 1995·Yeast·R B WicknerH K Edskes
May 13, 1997·Proceedings of the National Academy of Sciences of the United States of America·A BossersM A Smits

❮ Previous
Next ❯

Citations

Nov 19, 2003·Analytical Biochemistry·Laura MaxsonGerald S Baron
May 20, 1998·Journal of Chromatography. a·M J SchmerrA Jenny
Apr 11, 2001·Trends in Biochemical Sciences·B Caughey
Mar 6, 1999·Current Opinion in Microbiology·J Foster, N Hunter
Jul 3, 2009·Protein Engineering, Design & Selection : PEDS·Christina D OrrúByron Caughey
Feb 1, 2008·Annual Review of Pathology·Adriano AguzziMathias Heikenwaelder
Apr 14, 2007·BMC Biochemistry·Alan RigterAlex Bossers
Feb 12, 1998·Proceedings of the National Academy of Sciences of the United States of America·S K DebBurmanS Lindquist
Apr 16, 1998·Proceedings of the National Academy of Sciences of the United States of America·I L DerkatchS W Liebman
Jan 27, 2005·Biochemical and Biophysical Research Communications·Jurate KazlauskaiteTeresa J T Pinheiro
Jan 5, 2002·Journal of Autoimmunity·L SouanF Mor
Mar 31, 2012·Prion·Stacie J RobinsonChad J Johnson
Apr 9, 2002·Current Biology : CB·Nigel M Hooper
May 18, 2016·Brain Research·Zachary MonahanUdai Bhan Pandey
Mar 22, 2001·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·B Caughey
Mar 30, 2001·Cell Death and Differentiation·A Aguzzi, F L Heppner
Nov 21, 1998·The Journal of Biological Chemistry·B CaugheyR A Bessen
Jul 26, 2002·The Journal of Biological Chemistry·Ina Vorberg, Suzette A Priola
Jun 10, 1998·Journal of Clinical Microbiology·K I O'RourkeD P Knowles

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