Trial lecture: 'Non mammalian models (including cell-based) in the study of neurodegenerative diseases'
Time and place: June 11, 2024 10:15 AM, Nucleus, Bikuben, Kristine Bonnevies hus
Main research findings:
Huntington's disease (HD), a hereditary neurodegenerative disorder, provides a vital insight into the intricate interplay of genetics and neurobiology. Characterized by progressive motor dysfunction, cognitive decline, and psychiatric issues, HD is linked to genetic mutations that induce abnormal protein functions in the brain. My research leverages an enhanced mouse model engineered to mimic these human genetic alterations, offering a detailed profile of HD progression.
Through my study, we discovered significant insights using Fingolimod, a drug originally designed for Multiple Sclerosis, repurposed as a potential therapeutic for HD. Fingolimod acts on specific neural receptors to modulate neuroinflammatory responses, a common feature in neurodegenerative disorders. Administered over 30 weeks, Fingolimod not only slowed the deterioration of critical neuropathology but also ameliorated behavioral symptoms associated with HD. Notably, it reduced brain atrophy and body weight loss, highlighting its dual action on metabolic and neurodegenerative aspects of HD.
Additionally, Fingolimod demonstrated unexpected benefits in reducing testicular atrophy in male mice, suggesting its broader peripheral effects. Importantly, the treatment improved motor functions in a sex-dependent manner, reinforcing its potential as a targeted therapy. These findings underscore the viability of Fingolimod for further clinical trials, potentially offering novel treatment paradigms for HD and enhancing life quality for those affected by this challenging condition.
Adjudication committee
Prof. Joana Almeida Palha, School of Medicine, University of Minho, Braga
Prof. Lucía Chávez Gutiérrez, Department of Neurosciences, KU Leuven
Prof. Rafal Ciosk, Department of Biosciences, University of Oslo
Chair of defense
Prof. Kristian Gundersen, Department of Biosciences, University of Oslo
Supervisors
Prof. Jens Pahnke, Institute of Clinical Medicine, University of Oslo & Department of Pathology, Oslo University Hospital
Prof. Bernd Thiede, Department of Biosciences, University of Oslo
Consultant Pitt Niehusmann, Department of Neurosurgery, Oslo University Hospital
Consultant Henning Leske, Department of Pathology, Oslo University Hospital
Consultant Stefanie Marie Hansen, Department of Pathology, Oslo University Hospital
Assoc. Researcher Mirjam Brackhan, Instituto de Investigación Sanitaria IdiPaz