Jan Smeitink's purpose in life is finding a cure for mitochondrial diseases. Every year he sees hundreds of children with genetic mitochondrial defects in his clinic. The majority of the kids do not live to be adults. The energy factories of their cells, the mitochondria, fail to produce enough energy for other cellular processes. The result is a plethora of multisystem diseases. The IOP Genomics project has resulted in the development of a high-throughput screening platform for live cells using state-of-the-art imaging techniques. Hundreds of potential drugs for mitochondrial disorders can be tested using this platform. In the near future, this will lead to a cure for some of the mitochondrial diseases.
 

Aspergillus niger, well known as the fungus that produces black mold on old bread, forms an extended network of interconnected hyphae. This mycelium has an enormous capacity to secrete proteins and other compounds. Its productivity has led the food and pharmaceutical industry to use A. niger  as the preferred biological production factories for, among others, enzymes. New information about genetic signalling in the fungus cells has urged Marc van der Maarel to study the dynamics of the A. niger mycelium in more detail.

Not all old people are equally old. That is, their chronological age - the number of years they have lived - is not the same as their biological age - their life expectancy and health. Some people appear to have a hereditary taint for longevity. By studying a large group of very old people, who are obviously protected from ageing and disease, researchers are trying to find these genetic variations and the biomarkers by which they are expressed.

Mitochondrial disorders are a group of serious, often fatal diseases. Due to a genetic defect, the powerhouses of our cells are unable to produce enough energy from food. The mechanisms are poorly understood, let alone a therapy being available for patients. Jan Smeitink works to understand the energy metabolism in mitochondria by setting up a screening platform. This platform should generate enough knowledge to influence the energy metabolism and eventually cure disorders associated with it.

Prof. Dr. Arjen van Tunen stood at the cradle of the IOP Genomics programme at the dawn of the new millenium.  It was the first national Genomics research programme starting in the Netherlands. Van Tunen is content with the development of IOP Genomics since then, but urges the authorities to let go of traditional divisions: "THE industry and THE universities do not exist," van Tunen says. "Knowledge providers such as Keygene are in between. They should be acknowledged better for their merits and opportunities."
 

As one of two groups in the world scientists at the Hubrecht Laboratory succeeded to develop rat knock-outs. "What these people in Utrecht have done is very special," says Ruud Tegelenbosch of Noldus Information Technology, member of the industrial user group. "These animal models are very valuable in understanding gene functions in human health and disease and may also be useful for drug design and testing."