Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.

<h4>Background</h4>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...

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Autores principales: 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, Pierluigi Gambetti
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spelling oai:doaj.org-article:28fe48923f9445478b550bf34da116242021-11-25T06:26:37ZMultiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.1932-620310.1371/journal.pone.0008765https://doaj.org/article/28fe48923f9445478b550bf34da116242010-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20098730/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Background</h4>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.<h4>Methodology/principal findings</h4>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.<h4>Conclusions/significance</h4>Our 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.Silvio NotariFrancisco J MoleresStephen B HunterErmias D BelayLawrence B SchonbergerIgnazio CaliPiero ParchiWun-Ju ShiehPaul BrownSherif ZakiWen-Quan ZouPierluigi GambettiPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 5, Iss 1, p e8765 (2010)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
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
Pierluigi Gambetti
Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
description <h4>Background</h4>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.<h4>Methodology/principal findings</h4>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.<h4>Conclusions/significance</h4>Our 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.
format article
author 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
Pierluigi Gambetti
author_facet 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
Pierluigi Gambetti
author_sort Silvio Notari
title Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
title_short Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
title_full Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
title_fullStr Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
title_full_unstemmed Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States.
title_sort multiorgan detection and characterization of protease-resistant prion protein in a case of variant cjd examined in the united states.
publisher Public Library of Science (PLoS)
publishDate 2010
url https://doaj.org/article/28fe48923f9445478b550bf34da11624
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