The role of prion strains
The findings of the CIC bioGUNE researchers, who were part of an international team coordinated by the University of Kentucky, studied the role of the various properties of the prion strains in the transmission and spreading of a prion disease in depth, something which until now was unknown.
For this study, transgenic mice susceptible to prion disease in cervids were designed and generated. For the first time, these animals helped identify two biologically distinguishable strains of CWD prions, which has led to the conclusion that both prion strains are interrelated. Whilst the transgenic mice, designed to replicate Canadian deer prions, showed a stable propagation of each one of the prion strains, the transgenic mice designed to replicate prions from mule deer propagated an unstable mixture of CWD strains, preventing their separation through their biochemical properties.
The significance of this study is due to the fact that it is the first time that a single difference in the primary sequence of amino-acids of the protein responsible for prion diseases (Canadian deer versus mule deer) is shown, allowing the selection of prion strains. This discovery shows new data on the possibility of controlling the stability of some prion strains by means of modification of the primary structure of the protein responsible. For the first time, the study also shows the existence of at least two types of CWD strains with clearly distinct pathogenic behaviours.
In this last decade much has been achieved in the understanding of prions. In this regard, the epidemic of the bovine spongiform disease in Europe ("mad cow" dis
|Contact: Oihane Lakar|