Professor Adriano Aguzzi

International Awards

Hamdan Award for Medical Research Excellence - Emerging Viral & Prion Diseases
2003-2004
Professor Adriano Aguzzi was born in Bafia, Italy in 1960, and received university education in Switzerland and the USA. His latest medical studies were conducted at the Molecular Diseases Institute in Vienna in 1992.
Professor Adriano Aguzzi, who is at the Institute of Neuropathology (University of Zurich) investigates the molecular mechanisms of prion diseases, which include mad cow disease and Creutzfeldt-Jakob disease. 
 
In his first collaboration with Charles Weissmann, Aguzzi showed that the cellular prion protein, PrPC, is required for prion replication. In 1995-96, Aguzzi clarified an important question regarding the mechanism by which prions damage brain cells, by showing that expression of the normal prion protein is necessary for the development of damage.
Professor Aguzzi's interest then shifted to the mechanism by which prions reach the brain after entering the body. 
 
This problem is crucially important to public health, since prions typically enter the body from extracerebral sites, such as the gastrointestinal tract in the case of BSE and of new-variant CJD (which is thought to be caused by transmission of BSE from cows to humans).
 
Aguzzi demonstrated that neuroinvasion (the process by which prions travel through the body and reach the nervous system) relies on expression of the normal prion protein in non-hematopoietic extracerebral cells.
 
He proposed that neuroinvasion takes place in distinct steps: first the epithelial is trespassed, then the lymphoreticular system is colonized by the agent, and finally infectivity progresses from lymphoreticular organs to the central nervous system via peripheral nerves.
 
Professor Aguzzi then dissected the details of how prions subvert components of the immune system to gain access to the brain. In 1997, he demonstrated that B-Lymphocytes are required for the spread of the agent (a finding of importance for transfusion medicine). He went on to show that the presence of the normal prion protein on B-Lymphocytes is dispensable for neuroinvasion implying that prion replication within B-cells is unlikely to be important. In collaboration with Charles Weissmann, he then showed that the mechanism of action of B-lymphocytes consist of presentation of lymphotoxin-_ to follicular dendritic cells, and that inhibition of this signal transduction pathway can deplete lymphoreticular organs of prions. This discovery paved the way to post-exposure prophylaxis strategies exploiting soluble lymphotoxin-_ receptors.
 
Finally, Professor Aguzzi provided, in a seminal paper, proof-of-principle that neutralizing antibodies can protect against prions. This was achieved by expressing the heavy chain of a cloned anti-prion antibody as a transgene in the germ line of mice.
 
As a consequence, mice were protected against prion infection, yet did not develop autoimmune phenomena. These findings are extremely exciting, since they suggest that vaccines against prions may not be unrealistic.
 
From a therapeutic perspective, the most exciting discovery is that the modified prion protein attached to a human antibody fragment, when expressed in genetically modified mice, significantly hampered prion infection.
 
Professor Adriano Aguzzi has received several distinctions, including the Gold  of the European Molecular Biology Organization (EMBO), the Robert-Koch-Prize and the Ernst-Jung-Prize (among the highest German recognitions for life scientists), and honorary doctoral degrees from the University of Bologna (Italy) and from the University of Liege (Belgium).
 
In summary, Professor Adriano Aguzzi has produced over many years an impressive stream of fundamentally important, original contributions to the understanding of prion diseases. His discoveries have entered textbooks and are now finding their way in diagnostic tests as well as in human clinical trials.
 
It is for such achievements and contributions, and for his paper submitted that Professor Adriano Aguzzi deserves the Hamdan Award for Medical Research Excellence in the category of Emerging Viral and Prion Diseases, for the year 2003-2004.
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