Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States
| العنوان: | Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States |
|---|---|
| المؤلفون: | Stephen B. Hunter, Wun Ju Shieh, Piero Parchi, Wen-Quan Zou, Pierluigi Gambetti, Sherif R. Zaki, Silvio Notari, Francisco J. Moleres, Paul Brown, Ignazio Cali, Lawrence B. Schonberger, Ermias D. Belay |
| المساهمون: | Notari S., Moleres F.J., Hunter S.B., Belay E.D., Schonberger L.B., Cali I., Parchi P., Shieh W.J., Brown P., Zaki S., Zou W.Q., Gambetti P. |
| المصدر: | PLoS ONE PLoS ONE, Vol 5, Iss 1, p e8765 (2010) |
| سنة النشر: | 2010 |
| مصطلحات موضوعية: | Pathology, medicine.medical_specialty, Prions, Science, animal diseases, Blotting, Western, Public Health and Epidemiology/Infectious Diseases, Ovary, Biology, Creutzfeldt-Jakob Syndrome, 03 medical and health sciences, 0302 clinical medicine, Infectious Diseases/Prion Diseases, mental disorders, medicine, Humans, 030304 developmental biology, 0303 health sciences, Kidney, Gastrointestinal tract, Multidisciplinary, he study was supported by National Institutes of Health grant AG14359, Centers for Disease Control and Prevention (CCU515004), and the Charles S. Britton Fund to PG, Meninges, Brain, Virology, Immunohistochemistry, United States, 3. Good health, nervous system diseases, Neurological Disorders/Prion Diseases, medicine.anatomical_structure, Lymphatic system, grant NIH R01 NS062787 and the Creutzfeldt-Jakob Disease Foundation to WQZ, as for cost of supplies and salaries, Medicine, Histopathology, 030217 neurology & neurosurgery, Research Article, Peptide Hydrolases |
| الوصف: | BackgroundVariant Creutzfeldt-Jakob disease (vCJD) is a prion disease thought to be acquired by the consumption of prion-contaminated beef products. To date, over 200 cases have been identified around the world, but mainly in the United Kingdom. Three cases have been identified in the United States; however, these subjects were likely exposed to prion infection elsewhere. Here we report on the first of these subjects.Methodology/principal findingsNeuropathological and genetic examinations were carried out using standard procedures. We assessed the presence and characteristics of protease-resistant prion protein (PrP(res)) in brain and 23 other organs and tissues using immunoblots performed directly on total homogenate or following sodium phosphotungstate precipitation to increase PrP(res) detectability. The brain showed a lack of typical spongiform degeneration and had large plaques, likely stemming from the extensive neuronal loss caused by the long duration (32 months) of the disease. The PrP(res) found in the brain had the typical characteristics of the PrP(res) present in vCJD. In addition to the brain and other organs known to be prion positive in vCJD, such as the lymphoreticular system, pituitary and adrenal glands, and gastrointestinal tract, PrP(res) was also detected for the first time in the dura mater, liver, pancreas, kidney, ovary, uterus, and skin.Conclusions/significanceOur results indicate that the number of organs affected in vCJD is greater than previously realized and further underscore the risk of iatrogenic transmission in vCJD. |
| وصف الملف: | ELETTRONICO |
| اللغة: | English |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d0bc8ad75b978d9d2e965bf9e06e6d11Test http://hdl.handle.net/11585/83940Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....d0bc8ad75b978d9d2e965bf9e06e6d11 |
| قاعدة البيانات: | OpenAIRE |
| الوصف غير متاح. |