NR AWKK
AU Moleres,F.J.; Shieh,W.J.; Hunter,S.B.; Belay,E.D.; Schonberger,L.B.; Zou,W.Q.; Zaki,S.R.; Gambetti,P.
TI Variant Creutzfeldt-Jakob disease with detectable PrPsc in multiple organs and a novel PK-resistant PrPsc fragment in the brain
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions PR-23
PT Konferenz-Poster
AB Variant Creutzfeldt-Jakob Disease (vCJD) is a prion disease thought to be acquired by the consumption of prion-contaminated beef products. Worldwide, nearly 200 cases have been identified mainly in the United Kingdom. Two case-patients have been detected in the United States, although both were born and raised in Great Britain where they presumably acquired their prion infection. We have characterized the neuropathology and brain PrP profile in the first US vCJD case-patient whose duration of illness was unusually long, 32 months. We have also studied the presence of PrPsc in peripheral tissues by applying modifications to the sodium phosphotungstic acid (NaPTA) precipitation, in the attempt to increase the recovery of PrPsc. In addition to PrPsc seen in other vCJD patients, a novel N-terminally truncated, PK-resistant fragment of PrP was detected in all the brain regions examined, independent of the number of plaques present. With high PrPsc recovery, we have been able to detect PrPsc not only in the lymphoreticular system and the gastrointestinal tract, but also in skin, lungs, adrenal gland, kidney, urinary bladder, ovary, uterus and liver. Extraneural PrPsc glycotype was similar to that present in brain. Our preliminary results significantly expand the spectrum of organs affected in vCJD. If confirmed by further studies, these results could carry important implications with regard to iatrogenic transmission. In addition, our results show the presence of a novel, PK-resistant PrPsc fragment that may shed new light on the mechanisms of PrPsc formation in this disease. Supported by NIH grant AG14359; CDC Grant CCU 515004 and the Britton Fund. FJM is supported by a FPU grant 2003-5033.
AD Francisco J. Moleres, Wen-Quan Zou, Pierluigi Gambetti, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA; Wun-Ju Shieh, Ermias D. Belay (ebelay@cdc.gov), Lawrence B. Schonberger, Sherif R. Zaki, Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia 30333, USA; Stephen B. Hunter, Department of Pathology, Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, GA 30322, USA
SP englisch
PO Italien