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Related Experiment Videos

Prion diseases.

Richard T Johnson1

  • 1Pathology 627, The Johns Hopkins University School of Medicine & Bloomberg School of Public Health, Baltimore, MD 21287, USA. rtj@jhmi.edu

The Lancet. Neurology
|September 20, 2005
PubMed
Summary
This summary is machine-generated.

Prion diseases are fatal neurodegenerative disorders caused by infectious prion proteins. Transmission varies across human and animal diseases, complicating risk assessment and prediction.

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Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Molecular Biology

Background:

  • Prion diseases are fatal neurodegenerative disorders affecting the nervous system.
  • They are caused by a pathogenic isoform of the prion protein, a normal cell membrane constituent.
  • Creutzfeldt-Jakob disease (CJD) is the most common human prion disease.

Purpose of the Study:

  • To review the modes of transmission and risk factors associated with human and animal prion diseases.
  • To highlight the challenges in assessing and predicting the spread of prion diseases.
  • To understand the implications of prion protein's pathogenic isoform.

Main Methods:

  • Review of scientific literature on prion diseases.
  • Analysis of transmission routes in human and animal prionopathies.

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  • Examination of factors influencing disease spread and risk.

    • Main Results:

    • Human prion diseases include sporadic CJD, inherited forms, and iatrogenic transmission.
    • Consumption of infected material is linked to kuru and variant CJD.
    • Animal prion diseases like BSE and CWD are transmitted via contact or feed.

    Conclusions:

    • Prion disease transmission routes are diverse, including sporadic, inherited, iatrogenic, and consumption-based.
    • Animal prion diseases spread through direct contact or contaminated feed.
    • Variable transmission, species barriers, infectivity distribution, and strain variations complicate risk assessment and future predictions.