Dr. Jan Dobeš from the Department of Cell Biology at the Faculty of Science, Charles University, has received the prestigious Česká hlava award. He was awarded by the INVENTION prize for his outstanding achievement in the field of basic or applied research in the 23rd year of the competition.
In Finland, Norway or even in a small village in Sardinia, patients with a mutation of the Aire gene, which is crucial for the proper functioning of the immune system, are more frequently found. These patients suffer from autoimmune diseases as well as repeated yeast infections. Why, on the one hand, does the immune system over-attack its own body and cause diabetes and other autoimmune diseases, and on the other hand, is it unable to defend itself against yeasts that it would normally eradicate? We have finally managed to unravel this mystery that has remained unsolved for years.
Our research has shown that investigating rare diseases can lead to uncovering the basic principles of the immune system. Aire stands for autoimmune regulator, a protein that plays a vital role in training white blood cells in the thymus. In the thymus, white blood cells learn to distinguish between cells of their own body and harmful foreign invaders to which the immune system is supposed to respond. If the white blood cells make the mistake of reacting to their own cells, they are mercilessly destroyed to prevent the development of autoimmune diseases.
If the Aire gene is mutated, errors are introduced in this process. The white blood cells are not sufficiently trained and attack the body's own cells, leading to autoimmune diseases. However, this has been known before. But it has long remained a mystery why patients with the same mutation also suffer from frequent yeast infections that their immune systems cannot effectively fight.
Only our discovery has solved this mystery. We discovered another group of cells in the lymph nodes that also produce Aire. These cells are key to activating white blood responsible for fighting yeast infections. However, in patients with a mutation in Aire, the white blood cells do not receive the necessary signals that a yeast infection is growing in the body and so cannot take action against the infection, allowing the yeast infection to run rampant in the body.
Our research has not only shed light on one of the mysteries of immunology, but has also shown how the immune system fights yeast infections.
ABB's INVENTION Award is given for a discovery or an extraordinary achievement made in the last few years in the field of basic or applied research or for a technological innovation with a view to practical application.