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Related Experiment Videos

Prions in skeletal muscle.

Patrick J Bosque1, Chongsuk Ryou, Glenn Telling

  • 1Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143, USA.

Proceedings of the National Academy of Sciences of the United States of America
|March 21, 2002
PubMed
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Skeletal muscle in mice can propagate and accumulate prions, including the disease-causing prion protein (PrPSc). This finding is concerning for meat consumption and prion disease diagnosis in livestock and humans.

Area of Science:

  • Neuroscience
  • Pathology
  • Molecular Biology

Background:

  • Bovine spongiform encephalopathy (BSE) prions are linked to new variant Creutzfeldt-Jakob disease in humans.
  • Specified offals, rich in prions, are excluded from human food to prevent disease transmission.
  • The distribution and propagation of prions in non-neural tissues remain incompletely understood.

Purpose of the Study:

  • To investigate the capacity of skeletal muscle to propagate prions.
  • To determine if prions are produced or merely accumulate in muscle tissue.
  • To assess the implications of muscle prion accumulation for food safety and disease diagnosis.

Main Methods:

  • Wild-type mice were inoculated with specific strains of murine prions.
  • Prion titers and the presence of PrPSc were analyzed in various skeletal muscles.

Related Experiment Videos

  • Transgenic mice expressing prion protein (PrP) in muscle or liver were used to assess prion production versus accumulation.

    • Main Results:

    • High prion titers and PrPSc were detected in the skeletal muscle of inoculated mice.
    • Distinct muscles exhibited varying levels of PrPSc accumulation, with hind limb muscle showing the highest.
    • Transgenic mice showed de novo prion formation in muscle but low titers in liver, indicating tissue-specific factors influence prion tropism.

    Conclusions:

    • Skeletal muscle can intrinsically propagate and accumulate substantial prion titers.
    • Dietary exposure to prions via meat consumption is a potential risk, even without neural or lymphatic tissues.
    • Muscle tissue may serve as a diagnostic target for prion diseases in asymptomatic individuals.