Goodchild / De Strooper Lab – VIB/KULeuven – Belgium

Goodchild / De Strooper Lab

In September 2010, FDR donated 1 million euro to the ‘VIB Center for the Biology of Disease’ at the University of Leuven in Belgium, establishing an academic chair and a research team for investigating the pathophysiology of primary dystonia.

In addition to the 1 million euro from FDR, the ‘Flanders Institute for Biotechnology’ contributed 1 million euro to the initiative. This budget allows a research group to operate for at least five years.

Since January 2012, this research team is led by Prof. Dr. Rose Goodchild, under the directorship of Prof. Dr. Bart De Strooper.

The laboratory investigates dystonia pathophysiology by focusing on the molecular and cellular pathology of hereditary dystonia. But it also has the long-term goals of determining the neurological defects and identifying therapeutic targets to treat dystonia.

Currently, the laboratory explores the impact of the dystonia-causing mutations on protein and neuronal function, thereby trying to define the molecular and cellular ‘hits’ that cause dystonia. Prof. Goodchild has previously identified that the torsinA-protein–which is mutated in DYT1-patients–operates in the nuclear envelope lumen. Disease-associated ΔE-torsinA abnormally accumulates at this subcellular localization. The laboratory is continuing to explore torsinA function and dysfunction in the nuclear envelope, how this impacts nuclear envelope localized proteins and cellular activities, and how these contribute to neuronal development.

The laboratory has also initiated new projects that explore other forms of hereditary dystonia. These include DYT11 myoclonic dystonia caused by loss of epsilon-sarcoglycan (ESG). The lab is using biochemical, cell and mouse models to examine ESG normal function in neurons, and how loss of ESG function impacts developing neurons.