دورية أكاديمية

Multiorgan Detection and Characterization of Protease- Resistant Prion Protein in a Case of Variant CJD Examined in the United States

التفاصيل البيبلوغرافية
العنوان: Multiorgan Detection and Characterization of Protease- Resistant Prion Protein in a Case of Variant CJD Examined in the United States
المؤلفون: Silvio Notari, Francisco J. Moleres, Stephen B. Hunter, Ermias D. Belay, Lawrence B. Schonberger, Ignazio Cali, Piero Parchi, Wun-ju Shieh, Paul Brown, Sherif Zaki, Wen-quan Zou
المساهمون: The Pennsylvania State University CiteSeerX Archives
المصدر: ftp://ftp.ncbi.nlm.nih.gov/pub/pmc/3f/f7/PLoS_One_2010_Jan_19_5(1)_e8765.tar.gz
المجموعة: CiteSeerX
الوصف: Background: Variant 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 Findings: Neuropathological 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/Significance: Our results indicate that the number of organs affected in vCJD is greater than previously
نوع الوثيقة: text
وصف الملف: application/zip
اللغة: English
العلاقة: http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.356.7740Test
حقوق: Metadata may be used without restrictions as long as the oai identifier remains attached to it.
رقم الانضمام: edsbas.595EC887
قاعدة البيانات: BASE