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Updated: Aug 14, 2026

Assessing Transmissible Spongiform Encephalopathy Species Barriers with an In Vitro Prion Protein Conversion Assay
Published on: March 10, 2015
Pathological prion protein in muscles of hamsters and mice infected with rodent-adapted BSE or vCJD
Achim Thomzig1, Franco Cardone2, Dominique Krüger1
1Robert Koch-Institut (P24 - Transmissible Spongiform Encephalopathies), Nordufer 20, 13353 Berlin, Germany.
Abstract:
Recently, pathological prion protein (PrP(TSE)) was detected in muscle from sheep infected with scrapie, the archetype of transmissible spongiform encephalopathies (TSEs). This finding has highlighted the question of whether mammalian muscle may potentially also provide a reservoir for TSE agents related to bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob Disease (vCJD). Here, results are reported from studies in hamsters and mice that provide direct experimental evidence, for the first time, of BSE- and vCJD-associated PrP(TSE) deposition in muscles. Our findings emphasize the need for further assessment of possible public-health risks from TSE involvement of skeletal muscle.
Insights
Pathological prion protein (PrP(TSE)) was found in muscle tissue of animals with scrapie. This study provides the first experimental evidence of PrP(TSE) in muscle linked to bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob Disease (vCJD).
Area of Science:
- Neurology
- Veterinary Medicine
- Infectious Diseases
Background:
- Pathological prion protein (PrP(TSE)) detection in sheep muscle raises concerns about potential reservoirs for transmissible spongiform encephalopathies (TSEs).
- Previous findings suggest a possible link between TSE agents and mammalian muscle tissue.
Purpose of the Study:
- To investigate the presence of PrP(TSE) in muscle tissue associated with bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob Disease (vCJD).
- To provide experimental evidence of PrP(TSE) deposition in muscles relevant to BSE and vCJD.
Main Methods:
- Studies were conducted using hamster and mouse models.
- Experimental models were inoculated to assess PrP(TSE) deposition in skeletal muscle.
Main Results:
- Direct experimental evidence of PrP(TSE) deposition in muscles was observed in hamsters and mice.
- This study marks the first demonstration of BSE- and vCJD-associated PrP(TSE) in muscle tissue.
Conclusions:
- The findings confirm that skeletal muscle can harbor PrP(TSE) associated with BSE and vCJD.
- Further research is crucial to evaluate potential public health risks associated with TSEs in skeletal muscle.
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