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Prion infectivity in variant Creutzfeldt-Jakob disease rectum

J D F Wadsworth1, S Joiner, K Fox

  • 1MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.

Gut
|June 10, 2006
PubMed
Abstract

Insights

Variant Creutzfeldt-Jakob disease (vCJD) prions are infectious even at low levels of detectable prion protein (PrP(Sc)). This study demonstrates efficient prion transmission from vCJD rectal tissue, highlighting potential risks for surgical procedures.

Area of Science:

  • Neuroscience
  • Infectious Diseases
  • Biochemistry

Background:

  • Variant Creutzfeldt-Jakob disease (vCJD) is characterized by detectable prion protein (PrP(Sc)) in lymphoreticular tissues, unlike other human prion diseases.
  • Concerns exist regarding vCJD prion transmission during surgical procedures due to unknown asymptomatic infection prevalence.
  • The relationship between PrP(Sc) concentration and prion infectivity in vCJD remains uncharacterized.

Purpose of the Study:

  • To determine prion infectivity in vCJD tissues with minimal detectable PrP(Sc) levels.
  • To assess the potential for secondary transmission of vCJD prions.
  • To evaluate PrP(Sc) as a quantitative marker for prion infectivity.

Main Methods:

  • Utilized transgenic mice expressing human PrP (Tg(HuPrP129M(+/+)Prnp(o/o))-35 and Tg(HuPrP129M(+/+)Prnp(o/o))-45).
  • Inoculated mice with vCJD brain and rectal tissues, representing maximum and minimum detectable PrP(Sc) levels.
  • Assessed prion transmission efficiency in recipient mice.

Main Results:

  • Efficient prion infection transmission was achieved using vCJD rectal tissue.
  • Rectal tissue contained PrP(Sc) at concentrations significantly lower (10(4.7)-fold) than vCJD brain tissue.
  • Demonstrated infectivity in tissues with barely detectable PrP(Sc).

Conclusions:

  • Confirms the risk of vCJD prion secondary transmission through gastrointestinal procedures.
  • Supports the utility of PrP(Sc) as a reliable quantitative marker for vCJD prion infectivity.
  • Underscores the importance of stringent decontamination protocols for surgical instruments.

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