Körber European Science Award 2004 for physicians and molecular biologists / Therapies for a new group of hereditary diseases / Prize winners to do research in

Hamburg 30. 3. 2004 (PROTEXT/ots) - This year, the Körber-Stiftung in Hamburg has awarded the Körber prize including prizemoney of EUR 750,000 to an international team of physicians,biochemists and cell biologists wanting to do research oncongenital diseases of the glycoprotein metabolism in order todevelop new diagnostic procedures and therapies. Aninternational board chaired by the president of the Max-PlanckSociety, 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 theResearch Group), Prof. Dr. Markus Aebi, Institute forMicrobiology at the Swiss Technical University of Zurich, Prof.Dr. Thierry Hennet, Physiological Institute at the University ofZurich, Prof. Dr. Jaak Jaeken, department of Paediatrics at theUniversity Hospital Gasthuisberg in Louvain, Prof. Dr. LudwigLehle, Chair of Cell Biology and Plant Physiology at theUniversity of Regensburg and Prof. Dr. Gert Matthijs, Laboratoryfor Molecular Diagnosis at the Centre for Human Genetics inLouvain.

The diseases investigated by the prize winners are a group ofcongenital defects that have been recognised for less than 25years. They deal with the manufacture of a specific class ofprotein molecules - glycol-proteins - described as CongenitalDisorder of Glycosylation (CDG). Prevalence is similar to thatof 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 fromlimited physical mobility. About one-fifth of patients diewithin the first two years of their life from liver or renalfailure, cardiac insufficiency or infections. As the symptomsare however not as specific as in many other diseases, it is allthe more important to develop reliable methods of diagnosis - anaim of the research project. The prize winners were the first todescribe patients with CDG syndrome and they are among theworld's leading researchers of the disease.

In principle, the congenital disorders of the glycoproteinmetabolism are subject to the same biochemical mechanism: amongthe thousands of different protein molecules produced by eachsomatic cell, some also contain sugar - glycol-proteins. Theyplay a role, e.g. in the growth process, cellulardifferentiation, organ development, signal transmission,containment, infections or carcinogenesis. The cell producesthem by adding a sugar molecule to the protein molecule(glycosylation).

Aims of the research project:

The working groups of the Körber project coordinated by Kurtvon Figura aim to investigate why this process does not properlyfunction in CDG patients by experimenting with yeast cells andmice. 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 quitesimilar way to that in human cells, this organism is well suitedfor investigations. Using mutated yeast cells and genetically-engineered methods it may even be ascertained what triggers therespective genetic defect in humans.

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

The Körber Foundation seeks with its projects to engagecitizens actively in social discourses. In this sense it seesitself as a forum for new initiatives, providing opportunitiesfor involve-ment in politics, education, science andinternational communication in particular.

Active citizens can pass on knowledge, identify problems andinitiate activi- ties. With these kinds of stimulus theparticipants make an important con- tribution to the everydayculture of democracy.

Trustee Committee

Prof. Dr. Peter Gruss (Vors.), Prof. Dr. Sir Richard JohnBrook,

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.)

ots Original Text Service: Körber-Foundation Internet:http://www.presseportal.de

Further information: Dr. Nikolaus Besch

Kurt-A.-Körber-Chaussee 10 21033 Hamburg Telefon +49 40 72 50- 38 86 Telefax +49 40 72 50 - 36 45 E-Mailbesch@stiftung.koerber.de www.stiftung.koerber.de

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