High Titers of Transmissible Spongiform Encephalopathy Infectivity Associated with Extremely Low Levels of PrPSc in Vivo

@article{citeulike:2048564, abstract = {Diagnosis of transmissible spongiform encephalopathy (TSE) disease in humans and ruminants relies on the detection in post-mortem brain tissue of the protease-resistant form of the host glycoprotein PrP. The presence of this abnormal isoform (PrPSc) in tissues is taken as indicative of the presence of TSE infectivity. Here we demonstrate conclusively that high titers of TSE infectivity can be present in brain tissue of animals that show clinical and vacuolar signs of TSE disease but contain low or undetectable levels of PrPSc. This work questions the correlation between PrPSc level and the titer of infectivity and shows that tissues containing little or no proteinase K-resistant PrP can be infectious and harbor high titers of TSE infectivity. Reliance on protease-resistant PrPSc as a sole measure of infectivity may therefore in some instances significantly underestimate biological properties of diagnostic samples, thereby undermining efforts to contain and eradicate TSEs. 10.1074/jbc.M704329200}, author = {Barron, Rona M. and Campbell, Susan L. and King, Declan and Bellon, Anne and Chapman, Karen E. and Williamson, Anthony R. and Manson, Jean C. }, citeulike-article-id = {2048564}, doi = {http://dx.doi.org/10.1074/jbc.M704329200}, journal = {J. Biol. Chem.}, keywords = {prion}, month = {December}, number = {49}, pages = {35878--35886}, posted-at = {2007-12-03 09:24:20}, priority = {0}, title = {High Titers of Transmissible Spongiform Encephalopathy Infectivity Associated with Extremely Low Levels of PrPSc in Vivo}, url = {http://dx.doi.org/10.1074/jbc.M704329200}, volume = {282}, year = {2007} }

TY - JOUR ID - citeulike:2048564 L3 - citeulike-article-id:2048564 N2 - Diagnosis of transmissible spongiform encephalopathy (TSE) disease in humans and ruminants relies on the detection in post-mortem brain tissue of the protease-resistant form of the host glycoprotein PrP. The presence of this abnormal isoform (PrPSc) in tissues is taken as indicative of the presence of TSE infectivity. Here we demonstrate conclusively that high titers of TSE infectivity can be present in brain tissue of animals that show clinical and vacuolar signs of TSE disease but contain low or undetectable levels of PrPSc. This work questions the correlation between PrPSc level and the titer of infectivity and shows that tissues containing little or no proteinase K-resistant PrP can be infectious and harbor high titers of TSE infectivity. Reliance on protease-resistant PrPSc as a sole measure of infectivity may therefore in some instances significantly underestimate biological properties of diagnostic samples, thereby undermining efforts to contain and eradicate TSEs. 10.1074/jbc.M704329200 IS - 49 JF - J. Biol. Chem. EP - 35886 TI - High Titers of Transmissible Spongiform Encephalopathy Infectivity Associated with Extremely Low Levels of PrPSc in Vivo VL - 282 SP - 35878 KW - prion AU - Barron, Rona AU - Campbell, Susan AU - King, Declan AU - Bellon, Anne AU - Chapman, Karen AU - Williamson, Anthony AU - Manson, Jean PY - 2007/12/07/ UR - http://dx.doi.org/10.1074/jbc.M704329200 ER -