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Award
17.12.2018

Care-for-Rare Science Award for Dierk Niessing

Prof. Dr. Dierk Niessing from Helmholtz Zentrum München is honored with the Care-for-Rare Science Award for his contributions to research into the PURA syndrome. The structural biologist shares the 50,000 euro prize with Prof. Dr. med. Tobias Hirsch from Münster University Hospital.

Care-for-Rare Science Award for Prof. Dierk Niessing, Helmholtz Zentrum München and Prof. Tobias Hirsch, Münster University Hospital © BrauerPhotos / G.Nitschke fuer pro.movere

Dierk Niessing is head of a research group at the Institute of Structural Biology at Helmholtz Zentrum München and professor of Pharmaceutical Biotechnology at Ulm University. Together with his team, he is investigating the PURA syndrome, a hereditary neurodevelopmental disorder first described in 2014 that leads to epileptic seizures, among other symptoms. Currently, no treatment is available – conventional drugs have only limited or no effect (see also Background).

Niessing seeks to elucidate the molecular basis of the symptoms of this severe disorder. To achieve this, he contributes his expertise in structural biology and biochemistry, networks with other research disciplines (stem cell research and high-throughput analyses) and also includes a patient association (PURA Syndrome Foundation) in his work.

The Werner Reichenberger Foundation and the Care-for-Rare Foundation presented the Care-for-Rare Award to Dierk Niessing in a festive ceremony on December 14th in the  Alte Pinakothek in Munich. In his acceptance speech, Niessing said: “This confirmation of our research direction encourages us to continue along the chosen path and to investigate possible therapeutic approaches for the disease."

The award is also the result of successful cooperation within Helmholtz Zentrum München. "Without the collaboration with Micha Drukker's laboratory, for example, this research would not have been possible," said Niessing. In addition, three further groups from other disciplines are to join the project in the future. The teams led by Dr. Stefanie Hauck and Prof. Dr. Jerzy Adamski will approach the topic with proteome and metabolism studies. In addition, a biobank for PURA patient material will be established under the direction of Dr. Christian Gieger and Dr. Gabriele Anton.

Further Information

Background:
The PURA syndrome was first described in 2014 as a rare neurodevelopmental disorder and is caused by a mutation in the gene of the same name. It codes for the protein Pur-alpha, which serves as a transcription factor and RNA transport protein and fulfills various tasks in the cell. The symptoms resulting from mutations in the PURA gene include severe neurodevelopmental delay and various neonatal problems such as low blood pressure, respiratory problems, feeding difficulties or hypothermia. To date, around 250 affected patients have been identified, and a patient organization (PURA Syndrome Foundation) has been founded.

The Care-for-Rare Science Award is intended to promote scientific ideas and support scientists in initiating a research project in the field of rare diseases. Endowed with 50,000 euros, the prize is financed by the Werner Reichenberger Foundation and has been awarded annually since 2013 by the Care-for-Rare Foundation to promote innovative projects on rare diseases. The prize is embedded in the program Pro.movere – towards more movement: Under this motto, the Werner Reichenberger Foundation and the Care-for-Rare Foundation have created the Bavarian Founder‘s Award and the Care-for-Rare Science Award. The foundations want to highlight that society can be shaped and influenced in a decisive way by the social commitment of individual people.

As German Research Center for Environmental Health, Helmholtz Zentrum München pursues the goal of developing personalized medical approaches for the prevention and therapy of major common diseases such as diabetes mellitus, allergies and lung diseases. To achieve this, it investigates the interaction of genetics, environmental factors and lifestyle. The Helmholtz Zentrum München has about 2,500 staff members and is headquartered in Neuherberg in the north of Munich. Helmholtz Zentrum München is a member of the Helmholtz Association, a community of 19 scientific-technical and medical-biological research centers with a total of about 37,000 staff members. 

The Institute for Structural Biologyinvestigates the spatial structures of biological macromolecules, analyses their structure and dynamics and develops NMR-spectroscopy methods for carrying out these studies. Its goal is to illuminate the structural and molecular mechanisms and their role in disease. The structural data form the basis for the rational design and development of small molecular inhibitors in combination with chemical and biological approaches. http://www.helmholtz-muenchen.de/stb

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