Corinne Lasmezas, PhD
Professor, Department of Immunology and Microbial Science, Florida Campus
Scripps Research Joint Appointments Faculty, Graduate Program. Research Focus: Neurodegenerative diseases
Prion diseases are fatal neurodegenerative diseases affecting humans and animals. There is no cure for prion diseases. They are caused by an unconventional infectious agent called prion, which is thought to be composed mainly by a misfolded form of a host protein, the prion protein (PrP). In humans, prions cause Creutzfeldt-Jakob disease; in animals, scrapie has been known to ravage sheep flocks for two hundred years, and chronic wasting disease is of concern in the United States. The recent epidemic of bovine spongiform encephalopathy in the United Kingdom has caused major turmoil throughout Europe, and later, in other countries such as Japan, Canada and the United States, because the bovine prion disease is transmissible to humans causing variant Creutzfeldt-Jakob disease. The transmissibility of the latter by blood transfusion created a novel public health issue.
Recent years have witnessed major advances in our understanding of the role of the prion protein and other host factors in prion replication. Our aim now is to devise intervention strategies based on blocking the neurodegenerative process on one hand, and prion replication on the other hand. The latter is part of a drug discovery program that we are running in collaboration with the Scripps Florida Lead Identification Division.
Prion diseases belong to the same group of protein misfolding neurodegenerative diseases as Alzheimer, Parkinson, Huntington’s diseases, amyotrophic lateral sclerosis (ALS) and others. In all these diseases, a host protein misfolds, aggregates and becomes toxic to neurons. We use the prion protein as a model system and starting point to study the neurodegenerative mechanisms that cause these diseases. We aim to understand which form(s) of these culprit proteins cause the death of neurons, and to decipher the death program(s) that they trigger in order to devise neuroprotective strategies.