Neurodegenerative Disease Research (HAI-NDR)
Neurodegenerative diseases are a major cause of morbidity and the fifth-leading cause of death for people over 65 years of age. There is currently no cure for neurodegenerative diseases. A common feature of neurodegenerative diseases is the accumulation of misfolded cellular proteins in the nervous system that ultimately leads to neuronal death. Understanding the underlying molecular mechanisms and developing strategies to prevent or halt disease progression is a strategic priority for both HAI-NDR – the DZNE and the UofA.
Prion diseases are fatal infectious neurodegenerative diseases that occur in humans and animals. They share characteristic features with other neurodegenerative diseases and represent an ideal model to study basic principles of neurodegeneration. Prions are unconventional pathogens that consist of misfolded, aggregated prion protein (PrP). They lack coding nucleic acid and encipher their genetic information in protein structure that can be imposed on the normal cellular PrP. Different prion strains exist that preferentially propagate in distinct brain regions and cause disease after different incubation times. After decades of intense research, the exact structure of the infectious prion and how exactly strain information can be stored in protein structure remains unresolved.
The collaborative research activities of the HAI-NDR strive to address these unresolved questions by combining expertise in human and animal prion diseases with state of-the art technology in structural biology, cell biology, transcriptomics, and proteomics. The program focuses on two major themes:
The collaborative research projects are embedded in the successful ongoing research activities at both sites and include in vitro studies to in vivo models to patients.
An integral component of this research partnership between the Centre for Prions and Protein Folding Diseases (at UofA) and the German Center for Neurodegenerative Diseases (DZNE) is the exchange of students and trainees providing them with exposure and access to novel protocols, approaches and state-of-the-art instrumentation.
Image of mouse fibroblasts (nuclei stained in blue) taking up the misfolded, pathogenic prion protein (red)
Dr Ina Vorberg
Deutsches Zentrum für Neurodegenerative Erkrankungen (DZNE)
Dr Judd Aiken
Centre for Prions and Protein Folding Diseases
University of Alberta
Edmonton, Alberta, Canada