Pondering PRIONS: Infectious Proteins that Ravage Brains with Glenn Telling

One of the most fascinating stories in the history of science:  Cannibals in New Guinea; Mad Cows in England; CJD in humans; dying deer and elk in Colorado; and three Nobel Prizes. Once heretical, it is now well-established that certain infectious brain diseases can be caused not by a virus or bacteria, but by a protein we all have in our brains that goes rogue. The protein – cellular prion protein - transforms into a self-replicating PrPSc that destroy brain cells.  In this episode Professor Glenn Telling – Director of the Prion Research Center at Colorado State University – talks  about the science of prion disorders in humans and animals with a focus on chronic wasting disease in deer and elk.  Because prions in these animals have the potential to mutate into strains that can infect humans there is an urgent need to better understand the mechanisms of prion transformation and both intra- and inter-species transmission. Interestingly, the self-aggregating properties of pathogenic prions are very similar to those of amyloid and Tau proteins in Alzheimer’s disease, and alpha-synuclein in Parkinson’s disease. These similarities suggest a potential for the development of new therapeutic approaches applicable to all of these diseases.

LINKS

Professor Telling’s Labpage: https://labs.vetmedbiosci.colostate.edu/telling/

Related articles:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5874178/pdf/cshperspectmed-PRD-a024448.pdf

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3268957/pdf/prion0404_0252.pdf

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8109225/pdf/41582_2021_Article_488.pdf