Since its foundation around 10 years ago the VIVIT institute in Dornbirn and Feldkirch has been dealing with the impact of disorders of the sugar and lipid metabolisms on the pathogenesis of diseases such as atherosclerosis liver damage or coronary heart disease.
We take up amino acids with our food. They play a key role in almost all life processes in the human body. But what happens when the molecular machinery in the cells is no longer able to correctly process the amino acids? It was only about six years ago that Prof. Dr. Jörn Oliver Saß and his colleagues at the Freiburg University Medical Centre discovered a new metabolic disorder characterised by the defective degradation of a N-acetylated amino acids. Their research showed that this defect had similarities to Canavan disease, an inherited neurodegenerative disease. It is possible that other diseases are also associated with this defect. The Freiburg researchers are now investigating the underlying molecular relationships.
For many decades, a special low-protein (low-phenylalanine) diet has been the only way of treating children suffering from phenylketonuria (PKU) that effectively prevents them from developing severe impairments in mental function. However, this type of diet is not very tasty and it is often difficult to get children to cooperate. A paediatrician from Reutlingen, Prof. Dr. Friedrich Trefz, has now been able to show that the substance sapropterin is able to reactivate the defective metabolic pathway to a degree that means that some sufferers can return to a normal diet.
Most organisms need iron to survive, but too much iron is toxic, and can cause fatal organ failure. The same is true inside cells, where iron balance must also be maintained. In a study published today in Cell Metabolism, scientists at the European Molecular Biology Laboratory (EMBL) in Heidelberg, Germany, have discovered that a group of proteins named IRPs ensure that this iron balance is kept and as such are essential for cell survival. More specifically, they found that IRPs are required for the functioning of mitochondria, the cell’s energy factories.
The CEO of Genzyme, Henri Termeer, explains how a young company, “which had no ideas” managed to become an internationally successful company with 11,000 employees in around 40 countries: “Ideas that other people think you are crazy to have, are usually the ideas that are the most successful.” The company's subsidiary in the city of Constance has 27 employees.
Metabolic diseases are characterised by disorders of the intermediary metabolism which can either be inherited or acquired. The extent and incidence of metabolic diseases can differ considerably and can range from widespread diseases such as diabetes to rare congenital diseases that frequently have a fatal outcome. Through new approaches in genome research amongst other things science and industry are increasingly focusing on metabolic diseases.
What has the lipid metabolism of the human body got to do with inflammation? Scientists in Dr. Petra May’s group at the Centre for Neuroscience at the University of Freiburg recently found that molecules which normally regulate the availability of cholesterol and other water-insoluble substances, also interact with the signalling networks of the immune system. According to their findings, a receptor which mediates the uptake of certain lipoproteins into liver cells, helps to control the chaotic situations that can occur after inflammation.
Physicist Dr. Jan-Bernd Hövener makes magnetic resonance imaging devices smaller and their magnetic fields weaker in the hope that precisely these properties will help him detect abnormal metabolic processes and tumours. On 9th June 2015, the International Organisation for Medical Physics (IOMP) awarded Hövener the Young Scientist Award in Medical Physics, the organisation’s most important prize for up-and-coming scientists.
Microorganisms can be taught to do things that they are unable to do naturally. Bacterial metabolisms can be changed in order to make the bacteria produce new products and convert new substrates. In addition, existing bacterial conversion strategies can be optimised for biotechnological applications. Prof. Dr. Georg Sprenger is the head of the Institute of Microbiology at the University of Stuttgart and he is an expert in this field.
The supply of nutrients such as vitamins minerals and trace elements is directly related to the appearance of numerous metabolic diseases. In order to identify nutrient deficiencies in good time and counteract the development of diseases the Institute of Applied Biochemistry IABC in Kreuzlingen has developed investigation methods that enable statements to be made on an individuals personal nutrient requirements.
A research team led by microbiologist Dr. David Schleheck, in cooperation with chemists from the University of Konstanz, has discovered how sulpho-glucose is degraded in Escherichia coli bacteria. As this sulphurous glucose analogue is produced by all photosynthetically active organisms, the researchers’ discovery is of great importance for our understanding of the global sulphur cycle.
COPI vesicles, one of the three major types of intracellular transport vesicles, are an excellent example of how the molecular analysis of individual vesicle components can lead to an understanding of the biogenesis and transport mechanisms of membrane vesicles as a whole. The research carried out by Dr. Felix T. Wieland’s team at the University of Heidelberg has made a decisive contribution to such detailed insights.
Smog, chemicals in rivers, particular matter from copying machines in offices – human beings are exposed to many toxic influences. Environmental medical specialists are investigating the effect that these kinds of substances have at the same time as trying to find ways to reduce their influence on human health as much as possible. Molecular and cell biologists and even biotechnologists all have a key part to play in these efforts. On the one hand, they particpate in the investigation of the biological effects of environmental toxins on the cellular and molecular levels. On the other hand, they develop tools – so-called biosensors - for the early detection of environmental toxins.
Phenex Pharmaceuticals AG today announced the engagement of Prof. Dr. Dr. Werner Kramer, the former Head of Diabetes and Metabolic Diseases R&D at Sanofi Aventis, as a senior scientific advisor. At the end of last year, Prof. Kramer has left Sanofi Aventis after 25 years of very successful practice in drug discovery and development to devote himself to new scientific challenges. His long- and outstanding expertise in research and development of innovative drugs for the treatment of metabolic diseases is now available for Phenex ́ drug development programs.
In cooperation with the company Centogene AG Prof. Dr. Michael Przybylski from the Steinbeis Transfer Center for Biopolymer Analysis and Biomolecular Mass Spectrometry at the University of Konstanz has developed a method that involves a novel substance class and enables the parallel measurement of enzyme activity based on fluorometry and mass spectroscopy. The method for which the inventors have recently filed a patent application forms the basis for the development of rapid and highly specific enzyme test systems that allow the reliable and unequivocal diagnosis of diseases. In the interview with BIOPRO Prof. Przybylski gives us some insights into his invention.
The German Research Foundation will fund the “Molecular Diabetology and Endocrinology in Medicine” research training group established at the University of Ulm in 2004 for another four and a half years. According to information from the University, the grant totalling about 2.4 million euros in funding will be used to finance 20 new posts.
Scientists from the University Hospital of Heidelberg and the European Molecular Biology Laboratory (EMBL) have identified 20 new genes that are involved in the uptake of cholesterol into the cells. Twelve of these genes had previously been unknown. The findings are of great importance for the understanding of the regulation of cholesterol level and they make an important contribution to the treatment of coronary heart disease.
The latest results on the different metabolic pathways used by white and brown fat cells and potential transformations between the two cell types were discussed at the conference “Metabolism 2012: From Signalling to Disease” held on 15th and 16th November 2012 in Heidelberg. A special cell type, so-called “brite” adipocytes, may have the potential to be used in the treatment of obesity and related metabolic diseases such as diabetes.
Osteoporotic bones not only break very easily, they also heal badly when a fracture occurs. A fractured femur neck might mean that elderly patients have to remain bedridden for a long period and maybe even need nursing care. Fractures that heal badly, in combination with other diseases, frequently lead to a fatal outcome. A panregional team of researchers, including researchers from Ulm, has been working together since 2007 on basic research into fracture healing and bone regeneration in osteoporotic patients.
Thirty years ago cystic fibrosis CF formerly also known as mucoviscidosis was considered to be a disease that only affected children. Hardly any children with this hereditary metabolic disease ever reached early adulthood. Since then both the therapy and the life expectancy of CF patients have improved considerably. Dr. Gerd Döring from Tübingen is investigating the occurrence of respiratory tract defects that are common in CF patients.
Researchers led by the Ulm paediatrician Holger Cario have identified a rare, congenital genetic defect that leads to the dysregulation of the folic acid metabolism, resulting in aenemia, epilepsy and learning difficulties in children. All aspects of the disease can be treated quite successfully with the exception of the cramps associated with the defect.
Lysosomal storage diseases LSD are a group of around 50 rare inherited metabolic disorders. Only 12 LSDs have been described biochemically and microbiologically. Prof. Dr. Michael Przybylski from the Steinbeis Transfer Centre for Biopolymer Analysis and Proteomics at the University of Constance now hopes to change this situation. In a project carried out in cooperation with the biotech company Genzyme CEE Konstanz and the University of Timisoara Przybylski has developed two highly sensitive molecular methods involving mass spectrometry and fluorimetry and validated them for use in clinical diagnostics.
Starting today, Insilico Biotechnology will use Europe’s very fastest civil computer – going by the name of “Hermit“ – for its modeling and simulating processes. Hermit will cut computing time radically allowing metabolic predictions over several orders of magnitude. In the near future, predictions on the toxic effects of drugs across scales from cellular to organ level will be feasible.
Insilico Biotechnology is one of the partners in atransnational EU-project out to find a common, easy-to-use computer-based platform for modeling metabolic processes in organisms which are of interest for biotechnological applications. The main advantage of this approach is that it will shorten the time from concept to marketable European biotechnology product considerably for all involved.
A Freiburg research team has created the first complete map of special connections of nerve cells in zebrafish. The data identify all projection possibilities (“projectome”) of nerve cells in a particular class of messengers in the nervous system that is of great importance for medicine. The data were published in Nature Communications on 25th January.