Lack of prion infectivity in fixed heart tissue from patients with Creutzfeldt-Jakob disease or amyloid heart disease
| Autor: | Justin T. Cruite, Laura Solforosi, Michael B. A. Oldstone, Anne Ward, Young Pyo Choi, Suzette A. Priola, S. A. McCall, R. A. Williamson, Matthew J. Trifilo |
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| Rok vydání: | 2013 |
| Předmět: |
Amyloid
PrPSc Proteins Prions animal diseases Immunology Central nervous system Spleen Mice Transgenic Disease Biology Microbiology Creutzfeldt-Jakob Syndrome Mice Immune system Virology Cricetinae medicine Animals Humans Infectivity Amyloidosis Myocardium Brain medicine.disease Immunohistochemistry nervous system diseases Disease Models Animal medicine.anatomical_structure Insect Science Cardiomyopathies |
| Zdroj: | Journal of virology. 87(17) |
| ISSN: | 1098-5514 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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