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Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates

Prion diseases are transmissible fatal neurodegenerative disorders affecting humans and animals. A central step in disease progression is the accumulation of a misfolded form (PrP(Sc)) of the host encoded prion protein (PrP(C)) in neuronal and non-neuronal tissues. The involvement of peripheral tiss...

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Autores principales: Krasemann, Susanne, Neumann, Melanie, Geissen, Markus, Bodemer, Walter, Kaup, Franz-Josef, Schulz-Schaeffer, Walter, Morel, Nathalie, Aguzzi, Adriano, Glatzel, Markus
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2978702/
https://www.ncbi.nlm.nih.gov/pubmed/21085647
http://dx.doi.org/10.1371/journal.pone.0013906
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author Krasemann, Susanne
Neumann, Melanie
Geissen, Markus
Bodemer, Walter
Kaup, Franz-Josef
Schulz-Schaeffer, Walter
Morel, Nathalie
Aguzzi, Adriano
Glatzel, Markus
author_facet Krasemann, Susanne
Neumann, Melanie
Geissen, Markus
Bodemer, Walter
Kaup, Franz-Josef
Schulz-Schaeffer, Walter
Morel, Nathalie
Aguzzi, Adriano
Glatzel, Markus
author_sort Krasemann, Susanne
collection PubMed
description Prion diseases are transmissible fatal neurodegenerative disorders affecting humans and animals. A central step in disease progression is the accumulation of a misfolded form (PrP(Sc)) of the host encoded prion protein (PrP(C)) in neuronal and non-neuronal tissues. The involvement of peripheral tissues in preclinical states increases the risk of accidental transmission. On the other hand, detection of PrP(Sc) in non-neuronal easy-accessible compartments such as muscle may offer a novel diagnostic tool. Primate models have proven invaluable to investigate prion diseases. We have studied the deposition of PrP(Sc) in muscle and central nervous system of rhesus monkeys challenged with sporadic Creutzfeldt-Jakob disease (sCJD), variant CJD (vCJD) and bovine spongiform encephalopathy (BSE) in preclinical and clinical stage using biochemical and morphological methods. Here, we show the preclinical presence of PrP(Sc) in muscle and central nervous system of rhesus monkeys experimentally infected with vCJD.
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spelling pubmed-29787022010-11-17 Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates Krasemann, Susanne Neumann, Melanie Geissen, Markus Bodemer, Walter Kaup, Franz-Josef Schulz-Schaeffer, Walter Morel, Nathalie Aguzzi, Adriano Glatzel, Markus PLoS One Research Article Prion diseases are transmissible fatal neurodegenerative disorders affecting humans and animals. A central step in disease progression is the accumulation of a misfolded form (PrP(Sc)) of the host encoded prion protein (PrP(C)) in neuronal and non-neuronal tissues. The involvement of peripheral tissues in preclinical states increases the risk of accidental transmission. On the other hand, detection of PrP(Sc) in non-neuronal easy-accessible compartments such as muscle may offer a novel diagnostic tool. Primate models have proven invaluable to investigate prion diseases. We have studied the deposition of PrP(Sc) in muscle and central nervous system of rhesus monkeys challenged with sporadic Creutzfeldt-Jakob disease (sCJD), variant CJD (vCJD) and bovine spongiform encephalopathy (BSE) in preclinical and clinical stage using biochemical and morphological methods. Here, we show the preclinical presence of PrP(Sc) in muscle and central nervous system of rhesus monkeys experimentally infected with vCJD. Public Library of Science 2010-11-11 /pmc/articles/PMC2978702/ /pubmed/21085647 http://dx.doi.org/10.1371/journal.pone.0013906 Text en Krasemann et al. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
Krasemann, Susanne
Neumann, Melanie
Geissen, Markus
Bodemer, Walter
Kaup, Franz-Josef
Schulz-Schaeffer, Walter
Morel, Nathalie
Aguzzi, Adriano
Glatzel, Markus
Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates
title Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates
title_full Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates
title_fullStr Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates
title_full_unstemmed Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates
title_short Preclinical Deposition of Pathological Prion Protein in Muscle of Experimentally Infected Primates
title_sort preclinical deposition of pathological prion protein in muscle of experimentally infected primates
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2978702/
https://www.ncbi.nlm.nih.gov/pubmed/21085647
http://dx.doi.org/10.1371/journal.pone.0013906
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