Körber European Science Award 2004 for physicians and molecular biologists / Therapies for a new group of hereditary diseases / Prize winners to do research into defects in proteometabolism with yeast cells, mice and genetic engineering

Hamburg (ots) - This year, the Körber-Stiftung in Hamburg has awarded the Körber prize including prize money of EUR 750,000 to an international team of physicians, biochemists and cell biologists wanting to do research on congenital diseases of the glycoprotein metabolism in order to develop new diagnostic procedures and therapies. An international board chaired by the president of the Max-Planck Society, Prof. Dr. Peter Gruss, has selected the prize winners.

The prize winners:

Prof. Dr. Kurt von Figura, Department of Biochemistry II, Georg-August University of Goettingen (Coordinator of the Research Group), Prof. Dr. Markus Aebi, Institute for Microbiology at the Swiss Technical University of Zurich, Prof. Dr. Thierry Hennet, Physiological Institute at the University of Zurich, Prof. Dr. Jaak Jaeken, department of Paediatrics at the University Hospital Gasthuisberg in Louvain, Prof. Dr. Ludwig Lehle, Chair of Cell Biology and Plant Physiology at the University of Regensburg and Prof. Dr. Gert Matthijs, Laboratory for Molecular Diagnosis at the Centre for Human Genetics in Louvain.

The diseases investigated by the prize winners are a group of congenital defects that have been recognised for less than 25 years. They deal with the manufacture of a specific class of protein molecules - glycol-proteins - described as Congenital Disorder of Glycosylation (CDG). Prevalence is similar to that of other rare hereditary diseases such as chorea Huntington, haemophilia or vitreous bone disease.

Methods of diagnosis unexplored to date

CDG patients are often mentally retarded and suffer from limited physical mobility. About one-fifth of patients die within the first two years of their life from liver or renal failure, cardiac insufficiency or infections. As the symptoms are however not as specific as in many other diseases, it is all the more important to develop reliable methods of diagnosis - an aim of the research project. The prize winners were the first to describe patients with CDG syndrome and they are among the world's leading researchers of the disease.

In principle, the congenital disorders of the glycoprotein metabolism are subject to the same biochemical mechanism: among the thousands of different protein molecules produced by each somatic cell, some also contain sugar - glycol-proteins. They play a role, e.g. in the growth process, cellular differentiation, organ development, signal transmission, containment, infections or carcinogenesis. The cell produces them by adding a sugar molecule to the protein molecule (glycosylation).

Aims of the research project:

The working groups of the Körber project coordinated by Kurt von Figura aim to investigate why this process does not properly function in CDG patients by experimenting with yeast cells and mice. In particular, the prize winners would like to:

- find the molecular causes of CDG;

- use yeast cells to detect congenital defects;

- investigate the clinical picture in mice;

- develop methods of patient diagnosis;

- find new approaches to CDG therapies.

As the glycosylation process functions in yeast in a quite similar way to that in human cells, this organism is well suited for investigations. Using mutated yeast cells and genetically-engineered methods it may even be ascertained what triggers the respective genetic defect in humans.

In addition, the prize winners also want to study the clinical picture in mice. As their bodies are similar to that of humans, the use of animals is better than yeast to investigate how the symptoms of the disease materialize and how they may be healed. At present, it is possible to significantly alleviate the symptoms of two forms of CDG by giving patients those forms of sugar which their cells do not produce correctly. In other patients gene therapy procedures may be applied.

The Körber Foundation seeks with its projects to engage citizens actively in social discourses. In this sense it sees itself as a forum for new initiatives, providing opportunities for involve-ment in politics, education, science and international communication in particular.

Active citizens can pass on knowledge, identify problems and initiate activi-ties. With these kinds of stimulus the participants make an important con-tribution to the everyday culture of democracy.

Trustee Committee

Prof. Dr. Peter Gruss (Vors.), Prof. Dr. Sir Richard John Brook,

Prof. Dr. Olaf Kübler, Prof. Dr. Volker ter Meulen,

Prof. Dr. David N. Reinhoudt, Prof. Dr. Widmar Tanner,

Prof. Dr. Heinrich Ursprung, Prof. Dr. Sigmar Wittig

Executive Board

Christian Wriedt (Chair.)

Dr. Klaus Wehmeier (Dep. Chair.)

Dr. Wolf Schmidt

Board of Trustees

Ulrich Voswinckel (Chair.)