Prion Diseases Breakthroughs

Prion diseases are rare neurodegenerative diseases affecting 1 in every million people worldwide.  Some of the more common diseases are known as Creutzfeldt -Jakob disease in humans, or “Scrapie” in sheep and goats, Bovine Spongiform Encephalopathy (BSE) in cattle, and Chronic Wasting Disease in elk and mule deer.

Tanz Centre scientists have made some important discoveries in the field of prion diseases, including:

  1. In 2001 and 2004, Tanz researchers defined the antagonistic activities of PrP and Doppel in Tg mice.
  2. Inventing a cellular assay for the prion protein protective effect (2004).
  3. Demonstrating that the SPRN gene encodes a CNS expressed protein “shadoo” with biochemical resemblance to PrP. In functional assays in cerebellar neurons the protein has PrP-like protective activity against delta PrP or the Doppel protein.  Most remarkably, steady state levels of the shadoo protein are markedly reduced in prion infected animals. A simple hypothesis is that clinical target areas in prion disease reflect loss of shadoo’s protective action.