Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.

Prion infectivity and its molecular marker, the pathological prion protein PrP(Sc), accumulate in the central nervous system and often also in lymphoid tissue of animals or humans affected by transmissible spongiform encephalopathies. Recently, PrP(Sc) was found in tissues previously considered not...

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Main Authors: Achim Thomzig, Walter Schulz-Schaeffer, Arne Wrede, Wilhelm Wemheuer, Bertram Brenig, Christine Kratzel, Karin Lemmer, Michael Beekes
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2007-05-01
Series:PLoS Pathogens
Online Access:http://europepmc.org/articles/PMC1876502?pdf=render
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spelling doaj-095245e813094dbe87522181b8ad41642020-11-25T01:35:06ZengPublic Library of Science (PLoS)PLoS Pathogens1553-73661553-73742007-05-0135e6610.1371/journal.ppat.0030066Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.Achim ThomzigWalter Schulz-SchaefferArne WredeWilhelm WemheuerBertram BrenigChristine KratzelKarin LemmerMichael BeekesPrion infectivity and its molecular marker, the pathological prion protein PrP(Sc), accumulate in the central nervous system and often also in lymphoid tissue of animals or humans affected by transmissible spongiform encephalopathies. Recently, PrP(Sc) was found in tissues previously considered not to be invaded by prions (e.g., skeletal muscles). Here, we address the question of whether prions target the skin and show widespread PrP(Sc) deposition in this organ in hamsters perorally or parenterally challenged with scrapie. In hamsters fed with scrapie, PrP(Sc) was detected before the onset of symptoms, but the bulk of skin-associated PrP(Sc) accumulated in the clinical phase. PrP(Sc) was localized in nerve fibres within the skin but not in keratinocytes, and the deposition of PrP(Sc) in skin showed no dependence from the route of infection and lymphotropic dissemination. The data indicated a neurally mediated centrifugal spread of prions to the skin. Furthermore, in a follow-up study, we examined sheep naturally infected with scrapie and detected PrP(Sc) by Western blotting in skin samples from two out of five animals. Our findings point to the skin as a potential reservoir of prions, which should be further investigated in relation to disease transmission.http://europepmc.org/articles/PMC1876502?pdf=render
collection DOAJ
language English
format Article
sources DOAJ
author Achim Thomzig
Walter Schulz-Schaeffer
Arne Wrede
Wilhelm Wemheuer
Bertram Brenig
Christine Kratzel
Karin Lemmer
Michael Beekes
spellingShingle Achim Thomzig
Walter Schulz-Schaeffer
Arne Wrede
Wilhelm Wemheuer
Bertram Brenig
Christine Kratzel
Karin Lemmer
Michael Beekes
Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
PLoS Pathogens
author_facet Achim Thomzig
Walter Schulz-Schaeffer
Arne Wrede
Wilhelm Wemheuer
Bertram Brenig
Christine Kratzel
Karin Lemmer
Michael Beekes
author_sort Achim Thomzig
title Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
title_short Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
title_full Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
title_fullStr Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
title_full_unstemmed Accumulation of pathological prion protein PrPSc in the skin of animals with experimental and natural scrapie.
title_sort accumulation of pathological prion protein prpsc in the skin of animals with experimental and natural scrapie.
publisher Public Library of Science (PLoS)
series PLoS Pathogens
issn 1553-7366
1553-7374
publishDate 2007-05-01
description Prion infectivity and its molecular marker, the pathological prion protein PrP(Sc), accumulate in the central nervous system and often also in lymphoid tissue of animals or humans affected by transmissible spongiform encephalopathies. Recently, PrP(Sc) was found in tissues previously considered not to be invaded by prions (e.g., skeletal muscles). Here, we address the question of whether prions target the skin and show widespread PrP(Sc) deposition in this organ in hamsters perorally or parenterally challenged with scrapie. In hamsters fed with scrapie, PrP(Sc) was detected before the onset of symptoms, but the bulk of skin-associated PrP(Sc) accumulated in the clinical phase. PrP(Sc) was localized in nerve fibres within the skin but not in keratinocytes, and the deposition of PrP(Sc) in skin showed no dependence from the route of infection and lymphotropic dissemination. The data indicated a neurally mediated centrifugal spread of prions to the skin. Furthermore, in a follow-up study, we examined sheep naturally infected with scrapie and detected PrP(Sc) by Western blotting in skin samples from two out of five animals. Our findings point to the skin as a potential reservoir of prions, which should be further investigated in relation to disease transmission.
url http://europepmc.org/articles/PMC1876502?pdf=render
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