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Lack of prion infectivity in fixed heart tissue from patients with Creutzfeldt-Jakob disease or amyloid heart disease

Academic Article
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Overview

authors

  • Priola, S. A.
  • Ward, A. E.
  • McCall, S. A.
  • Trifilo, M.
  • Choi, Y. P.
  • Solforosi, L.
  • Williamson, R. A.
  • Cruite, J. T.
  • Oldstone, Michael

publication date

  • September 2013

journal

  • Journal of Virology  Journal

abstract

  • In most forms of prion disease, infectivity is present primarily in the central nervous system or immune system organs such as spleen and lymph node. However, a transgenic mouse model of prion disease has demonstrated that prion infectivity can also be present as amyloid deposits in heart tissue. Deposition of infectious prions as amyloid in human heart tissue would be a significant public health concern. Although abnormal disease-associated prion protein (PrP(Sc)) has not been detected in heart tissue from several amyloid heart disease patients, it has been observed in the heart tissue of a patient with sporadic Creutzfeldt-Jakob Disease (sCJD), the most common form of human prion disease. In order to determine whether prion infectivity can be found in heart tissue, we have inoculated formaldehyde fixed brain and heart tissue from two sCJD patients, as well as prion protein positive fixed heart tissue from two amyloid heart disease patients, into transgenic mice overexpressing the human prion protein. Although the sCJD brain samples led to clinical or subclinical prion infection and deposition of PrP(Sc) in the brain, none of the inoculated heart samples resulted in disease or the accumulation of PrP(Sc). Thus, our results suggest that prion infectivity is not likely present in cardiac tissue from sCJD or amyloid heart disease patients.

subject areas

  • Amyloidosis
  • Animals
  • Brain
  • Cardiomyopathies
  • Creutzfeldt-Jakob Syndrome
  • Cricetinae
  • Disease Models, Animal
  • Humans
  • Immunohistochemistry
  • Mice
  • Mice, Transgenic
  • Myocardium
  • PrPSc Proteins
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Identity

PubMed Central ID

  • PMC3754135

International Standard Serial Number (ISSN)

  • 0022-538X

Digital Object Identifier (DOI)

  • 10.1128/jvi.00692-13

PubMed ID

  • 23785217
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Additional Document Info

start page

  • 9501

end page

  • 9510

volume

  • 87

issue

  • 17

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