CIC bioGUNE research team

The Prion Lab of the Proteomics Research Unit belonging to the Centre for Cooperative Research in Biosciences - CIC bioGUNE -, has been recently inaugurated and is located in Building No. 502 in the Bizkaia Technology Park. The laboratory is committed to a thorough study of transmissible spongiform encephalopaties (TSEs), like the so called mad cow disease, among others.

The main research line focuses on the study of molecular mechanisms leading to interspecies prion transmission. Researchers aim at understanding the ways by which prions can infect some species and not others. The study and design of new molecules blocking prion replication amenable to be used in therapeutic treatments are inherent activities of this research team.

“Prions are pathogens causing TSEs, also known as prion diseases. TSEs belong to the fatal neurodegenerative diseases group, which affect both human beings and animals and for which there is no available therapy. The origin of these diseases is varied: hereditary, sporadic (in theory, spontaneous) and infectious”, reports Dr. Castilla, Head of the Prion Lab.

The epidemic of the bovine transmissible spongiform encephalopathy, so-called mad cow disease, in Europe triggered the number of research teams dealing with this kind of infectious agents. In consequence, the last decade has witnessed major advances in prion’s knowledge. However, there is still much to be discovered.

According to Castilla “prions probably are one of the most intriguing pathogens in nature, as their theoretical single protein composition and the presence of clearly different strains give them an unprecedented scientific value. Their replication mechanism, which resembles that of Alzheimer or Parkinson diseases, among others, makes them unique pathogens. In addition, as the prion nature is still a mystery, many researchers, including those in my team, find this study completely irresistible".

Scrapie, a fatal degenerative disease in sheep and goats, is probably the most ancient prion disease. However, the BSE (Bovine Spongiform Encephalopathy) has drawn much more public attention for being the cause of a new human disease and for its proved transmission to other species. Besides, the Creutzfeldt Jacob disease, even with a very low annual incidence of 1-2 cases per million inhabitants, is the big unknown, especially in sporadic cases whose origin is still a mystery. In the Basque Country the situation is quite uncommon, as there is a significant percentage of the global cases of fatal familial insomnia, a prion human disease of genetic origin.