Dorothee Dormann honored for dementia research

Munich, 02/24/2014

LMU biochemist Dorothee Dormann has won the Heinz Maier-Leibnitz Prize, viewed as the most distinguished German award available specifically to junior researchers, for her work on the molecular pathogenesis of neurodegenerative diseases.

Biochemist Dorothee Dormann studies the molecular mechanisms that underlie the development of frontotemporal dementia (FTD). The incidence of FTD among dementia patients over the age of 65 is second only to that of Alzheimer’s disease. The condition is characterized by ongoing loss of nerve cells primarily in the frontal lobes of the brain, and is associated with drastic changes in personality and social behavior. As with Alzheimer’s and other neurodegenerative illnesses, the formation of insoluble aggregates containing specific proteins plays a crucial role in nerve-cell dysfunction and death.

The proteins found in the FTD-associated aggregates, called TDP-43 and FUS, normally act in the cell nucleus. There they play an important role in regulating the processing of the RNA blueprints that program protein synthesis, which are transcribed from the nuclear DNA before being exported to the cytoplasm. In patients suffering from FTD, however, the two proteins accumulate and form aggregates in the cytoplasm, and never get to the nucleus. The block in uptake could lie in the molecular machinery responsible for their import into the nucleoplasm. Alternatively, as Dormann has already shown in one particular case, the proteins could lack the structural features that normally serve to direct them to the nucleus. This is why Dorothee Dormann, who leads a research group at LMU’s Institute of Metabolic Biochemistry (headed by Professor Christian Haass), focuses on defining what exactly prevents the uptake of proteins like TDP-43 and FUS into the cell nucleus.

Dormann’s findings not only shed light on the pathogenesis of FTD, they are also relevant to other neurodegenerative diseases, particularly amyotrophic lateral sclerosis (ALS). Researchers believe that ALS is closely related to FTD at the molecular level, even though the two conditions differ markedly from each other in their clinical manifestations. ALS patients show progressive loss of voluntary muscle function, which rapidly leads to loss of the ability to speak and to swallow. As a result, patients succumb to the illness within a few years of diagnosis. The typical symptoms are caused by the specific degeneration of the motor nerves that control the voluntary muscles, but aggregates containing TDP-43 and FUS turn out to play a central part in this process also.

Dorothee Dormann currently works at LMU’s Adolf Butenandt Institute, but will soon move to the Institute of Cell Biology (which is part of the Medical Faculty) to lead an Emmy Noether Junior Research Group, financed by the Deutsche Forschungsgemeinschaft (DFG). Born in 1976, Dormann studied Biochemistry at Tübingen University, and obtained her doctorate at Rockefeller University in New York, before joining LMU as a postdoctoral fellow in 2007.

The Heinz Maier-Leibnitz Prize, funded by the Federal Ministry of Education and Research (BMBF), is awarded annually by the DFG to ten young researchers who have produced outstanding work. The Prize is worth 20,000 euros.

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