Susanne Knorr, Senior Consultant Neurologist and Researcher Würzburg University Hospital. Photo by Stephan Röhl
Our congratulations to Dr. Susanne Knorr, department of Neurology, University Hospital of Würzburg, Germany and the David Marsden Award 2022 winner for her paper:
“The evolution of dystonia-like movements in TOR1A rats after transient nerve injury is accompanied by dopaminergic dysregulation and abnormal oscillatory activity of a central motor network.”
Dr. Knorr was presented with the award by Dystonia Europe (DE) President Adam Kalinowski at the Dystonia Europe 29th Annual Conference and Dystonia Days 2022 in Copenhagen, Denmark last May.
Dr. Knorr expressed her thanks to the judging panel, DE and Ipsen for selecting her paper. She then presented her work.
About the winner:
Dr. Knorr is an expert in basic research of movement disorders. She studied biology at the University of Würzburg. In 2015, she started her PhD thesis in the field of dystonia in the lab of Chi Wang Ip and Jens Volkmann.
Currently she is a postdoctoral research associate in the experimental movement disorder group of Chi Wang Ip.
During her many years of experience as a technician and a biologist in basic research, she gained a wide range of knowledge in molecular biology, biochemistry, behavior analysis, animal stereotactic surgeries, and experimental Deep Brain Stimulation (DBS).
About the research:
Basic research is important to deepen our understanding of disease pathomechanisms and finding new therapy strategies. For this, the use of a suitable disease model is crucial.
In dystonia research, animal models are highly relevant, because it is assumed that the network of the brain is affected by the disease and therefore we need a model comprising different cell populations, different brain structures and interconnections, which cannot be guaranteed in petri dish experiments. However, for DYT-TOR1A dystonia no animal model exists that reflects every aspect of the disease pathology. Either the DYT-TOR1A models are genetically modified animals without showing a dystonic phenotype, or the animals show a dystonic phenotype without a genetic background. Our aim was to generate an animal model, which mimics human dystonia by combining a known genetic dystonia background with a dystonic phenotype. In the genetically modified DYT-TOR1A rat model ∆ETorA, which expresses the human mutant TOR1A protein, a crush injury of the right sciatic nerve was implemented as an environmental factor to trigger a dystonic phenotype. Behavioral analysis revealed dystonia-like movements in the nerve-injured ∆ETorA rats. Further analysis of this model showed abnormalities in the striatal dopamine metabolism as well as alteration of theta and beta oscillations in the brain network of nerve-injured ∆ETorA rats. In addition, we could demonstrate that deep brain stimulation in dystonic ∆ETorA rats reduced dystonia and normalized pathologic brain network oscillations. This study forms the foundation for further analysis of pathomechanisms and treatment strategies by providing a new DYT-TOR1A rodent model.