Researching the genetic causes of infectious and congenital immunodeficiency disorders

This year the Robert Koch Foundation’s €100,000 Robert Koch Award is being equally divided between Professors Jean‐Laurent Casanova from Rockefeller University / Howard Hughes Medical Institute, New York, USA, and Alain Fischer from the Collège de France and Imagine Institute, Hôpital Necker‐Enfants‐Malades, Paris, France. The two scientists are being honored for their groundbreaking work on understanding host genes and their products in infectious diseases. Emeritus Professor Hermann Bujard from the Centre for Molecular Biology at Heidelberg University is being awarded the Robert Koch Gold Medal for his life’s work, in particular for his research on malaria infections.

The award ceremony will take place on November 14, 2014 at the Berlin‐ Brandenburg Academy of Sciences and Humanities.

“Holes” in the immune defense
Professor Jean‐Laurent Casanova heads the St. Giles Laboratory of Human Genetics of Infectious Disease at Rockefeller University, New York, USA. The pediatric immunologist pursued the question as to why some children develop severe infectious diseases after coming into contact with certain pathogens, while most other children do not. In his pioneering research, Casanova and his colleagues discovered “holes” in the immune systems of otherwise healthy children that make them susceptible to specific, sometimes life‐threatening infectious diseases. These “holes” are caused by congenital mutations in a single gene, and are responsible for the susceptibility to certain infections including mycobacterial diseases, herpes simplex virus encephalitis, invasive pneumococcal disease, chronic mucocutaneous candidiasis, or Kaposi sarcoma.

The practical implementations of these findings include molecular diagnosis and genetic counselling for the patients and their families as well as the development of targeted therapies with recombinant cytokines aimed at restoring a deficient immune response.

The second award winner, Professor Alain Fischer, who is the Director of the Research Institute for Genetic Diseases at the Hôpital Necker‐Enfants Malades, Paris, France, is also involved in discovering “holes” or switching errors in the immune system – congenital immune defects. As a result of his groundbreaking research work and the gene therapy method developed by him, he is considered one of the pioneers in this field. For example, the pediatric immunologist was the first scientist to successfully deploy gene therapy with young patients with severe combined immunodeficiency (SCID gc).

In addition to gene therapy and the genetics of immunological disorders, the main focus of his work is concerned with researching the lymphoid system, which plays an important role in the immune defenses against pathogens.

Focus on malaria vaccines
Research into mechanism which control the activity of genes as well as their practical implementation for combating infectious diseases form the main research areas of Emeritus Professor Hermann Bujard from the Centre for Molecular Biology at Heidelberg University, who is being honored with the Robert Koch Gold Medal for his life’s work. As head of biological research at Hoffmann‐La Roche, he already developed a preliminary research program for developing a vaccine against malaria back in 1983. At the end of 1985, Bujard became director for the further development of the today world‐renowned Centre for Molecular Biology (ZMBH) in Heidelberg.

There, in collaboration with West African colleagues, Bujard continues his research into a malaria vaccine, which will shortly undergo clinical testing. The "gene switches" developed in the Bujard laboratory have found wide application in biomedical research, in the development of new drugs against AIDS and hepatitis B, as well as novel strategies to combat insect‐borne infectious diseases, such as dengue fever currently proliferating worldwide.

About the Robert Koch Foundation
The Robert Koch Foundation is a non‐profit foundation dedicated to the promotion of medical progress and is based in Berlin. It promotes basic scientific research in the field of infectious diseases, as well as exemplary projects that address medical and hygienic issues. Patron of the Foundation, which was founded in 1907, is German President Joachim Gauck.

The Foundation confers a number of distinguished scientific awards each year: the Robert Koch Award – one of Germany's most distinguished scientific awards, the Robert Koch Gold Medal, three awards for young scientists and, for the first time in 2013, the Hospital Hygiene and Infection Prevention Award.

Robert Koch (1843 – 1910), after whom the award is named, was the founder of modern‐day bacteriology, for which he was awarded the 1905 Nobel Prize for Medicine and Physiology. From 1891 until his retirement in 1904, Koch was Head of the Institute for Infectious Diseases in Berlin.

26.06.2014