Disease-related prion protein is readily detectable in lymphoreticular tissues in variant Creutzfeldt-Jakob disease.
However, this is not so in other forms of human prion disease.
The unknown population prevalence of asymptomatic Creutzfeldt-Jakob disease infection has led to concerns that secondary transmission of Creutzfeldt-Jakob disease prions will occur through surgical procedures.
To date PrPSc:prion infectivity ratios have not been determined in Creutzfeldt-Jakob disease.
It is unknown whether Creutzfeldt-Jakob disease prions are similar to experimental rodent prions, where disease-related prion protein concentration typically reflects infectious prion titer.
|Tissues contain the maximum and minimum levels of detectable prions|
Professor Colling and colleagues from England investigated prion infectivity in Creutzfeldt-Jakob disease tissue containing barely detectable levels of disease-related prion protein.
Transgenic mice expressing only human prion proteins were inoculated with brain or rectal tissue from a previously characterised patient with Creutzfeldt-Jakob disease.
The investigative team noted that these tissues contain the maximum and minimum levels of detectable disease-related prion protein that have been observed in Creutzfeldt-Jakob disease.
The team observed efficient transmission of prion infection in transgenic mice inoculated with Creutzfeldt-Jakob disease rectal tissue containing prion protein at a concentration of 105-fold lower than that in Creutzfeldt-Jakob disease brain.
Professor Colling's team concludes, “These data confirm the potential risks for secondary transmission of Creutzfeldt-Jakob disease prions via gastrointestinal procedures and support the use of disease-related prion protein as a quantitative marker of prion infectivity in Creutzfeldt-Jakob disease tissues.”