(pd/res) The Körber Foundation in Hamburg has awarded this year's Körber Prize (1), which carries € 750,000 prize money, to an international team of physicians, biochemists and cell biologists who want to research the congenital disease of glycoprotein metabolism in order to develop new methods for diagnosis and therapies. An international board of trustees under the guidance of the President of the Max-Planck Society, Professor Peter Gruss, chose the winners, of whom Professor Markus Aebi from the Institute of Microbiology at ETH Zurich is one. The award winning team comprises Professor Thierry Hennet from the Physiological Institute of the University of Zurich, Kurt von Figura from the Faculty of Biochemistry II of the Georg-August University in Göttingen (research team co-ordinator), Jaak Jaeken from the Department of Paediatrics of the University Hospital Gasthuisberg in Leuven, Ludwig Lehle from the Chair of Cell Biology and Plant Physiology at the University of Regensburg and Gert Matthijs from the Laboratory for Molecular Diagnosis at the Center for Human Genetics in Leuven.

The disease that the team is researching is actually a group of hereditary defects, whose existence has been known for less than 25 years. They involve a certain class of protein molecules – the glycoproteins – that are known collectively as Congenital Disorder of Glycosylation (CDG). The frequency with which such disorders occur is approximately the same as other hereditary diseases, such as Huntington's chorea, haemophilia or osteogenesis imperfecta.

Unexplored methods of diagnosis

People suffering from CDG are often mentally retarded and they have are limited in their physical mobility. Around a fifth die very early on, within two years of birth, of liver or renal insufficiency, weak heart muscles or from infections. Because none of the symptoms are disease specific, as they are in many other illnesses, reliable methods to diagnose CDG are urgently required. This is an aim of the research project. The winners of the prize were the first to identify and describe people with the CDG syndrome and they are among the best in the world when it comes to researching this group of diseases.

Markus Aebi, Professor of Mycology, one of the winners of the Körber Prize. large

Contribution of ETH Zurich

Markus Aebi (2): "Our contribution to the project is yeast cells." ETH Life reported on the matter in an article (3) and it was also the subject of the Bulletin in April 1999. One of the numerous steps that regulates glycosylation is defect in people who have CDG syndrome. Aebi explains, " We – that is the research team that won this year's prize – were able to make use of the fact that, initially, the glycosation process unfolds in yeast and human beings in the same way. We examined the structure of the oligo-saccharides in cells that had been taken from CDG patients and compared the results of the analysis with yeast mutant stems that also showed a defect in this biosynthesis. Using such comparisons we are able to identify the molecular cause of such a hereditary CDG disease.

Aim of the research project

The team, under continuing co-ordination from Kurt von Figura, now wants to use the yeast cells not only to discover the origins of other congenital defects, but also to find new approaches for therapies.