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Updated: May 10, 2026
10:12
Protein Misfolding Cyclic Amplification of Prions
Published on: November 7, 2012
BSE-associated prion-amyloid cardiomyopathy in primates
Susanne Krasemann1, Giulia Mearini, Elisabeth Krämer
1University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Emerging Infectious Diseases
|June 6, 2013
Summary
Bovine spongiform encephalopathy prions can cause heart amyloidosis in macaques, leading to cardiac distress. This finding may explain cardiac symptoms in variant Creutzfeldt-Jakob disease patients.
Area of Science:
- Veterinary Medicine
- Neuropathology
- Cardiology
Background:
- Prion diseases, like bovine spongiform encephalopathy (BSE), are fatal neurodegenerative disorders.
- BSE prions are known to cause spongiform encephalopathy in cattle and variant Creutzfeldt-Jakob disease (vCJD) in humans.
- Cardiac involvement in prion diseases is not well-established.
Purpose of the Study:
- To investigate the potential for prion amyloidosis in the heart following experimental BSE prion inoculation in macaques.
- To explore the association between cardiac pathology and clinical signs of cardiac distress in a primate model of prion disease.
Main Methods:
- Three macaques were intraperitoneally inoculated with BSE prions.
- Clinical signs, disease duration, and pathological findings, including cardiac tissue, were monitored and analyzed.
Main Results:
- One of the three macaques developed prion amyloidosis in the heart.
- This macaque exhibited a prolonged disease duration and clear signs of cardiac distress.
- Prion-amyloid cardiomyopathy was identified as the likely cause of cardiac symptoms.
Conclusions:
- Experimental BSE prion infection in macaques can lead to cardiac prion amyloidosis.
- Prion-amyloid cardiomyopathy may be a contributing factor to cardiac manifestations in variant Creutzfeldt-Jakob disease.
- This study highlights the potential for systemic prion spread and cardiac pathology in affected individuals.
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