Aguzzi’s and Weissmann’s groundbreaking discoveries on the mechanisms underlying prion diseases have major implications for the understanding of other neurodegenerative conditions, including Alzheimer’s and Parkinson’s disease. The prize is awarded by the Piepenbrock Group and the German Center for Neurodegenerative Diseases (DZNE). The award ceremony will be held in Berlin on September 24, 2013.
This year the Hartwig Piepenbrock-DZNE prize has a special significance. It marks the company's 100th anniversary and therefore honors the two winners with 100,000 Euro. But 2013 is also the year where special remembrance is given to Hartwig Piepenbrock who forged this company into one of the largest German family firms. He was diagnosed with dementia several years ago and died on July 3. For many years, he was a great benefactor of the arts, science and society. The prize, sponsored by the Piepenbrock Group, recognizes the outstanding research achievements of Profs. Weissmann and Aguzzi. The winners have been selected by an international panel including highly recognized scientists and representatives from the DZNE and the Piepenbrock family.
"The outstanding scientific contributions of Charles Weissmann and Adriano Aguzzi are of extraordinary importance for the entire research field of neurodegenerative diseases," comments Prof. Johannes Dichgans, chairman of the selection committee. "With their prion research they have unveiled previously unknown mechanisms that are crucial for many of these diseases, including Alzheimer’s disease and Parkinson’s disease”. A fundamental aspect of these mechanisms is the accumulation of endogenous, misfolded proteins. The work of Aguzzi and Weissmann provides a solid basis to design new strategies for early diagnosis as well as effective prevention and therapy.
The initial work from Weissmann and Aguzzi provided the most convincing evidence that in prion diseases like BSE or Creutzfeld-Jakob disease an endogenous protein is of central importance. They showed that infective material causes nerve cell death only if this particular protein is present. In subsequent work, the research teams led by Aguzzi and Weissmann clarified fundamental mechanisms by which misfolded proteins, such as prions, could cause toxicity. Work from the Weissmann lab has led to the development of new assays for prion replication and toxicity, as well as characterizing sources of infectivity in humans. Aguzzi and his team went on to reveal how prions propagate from cell to cell and finally invade the brain.
Accumulation of endogenous, misfolded proteins is also at the core of other neurodegenerative disorders including Alzheimer's and Parkinson's diseases. Although the cause of misfolded protein accumulation is not an infection as in some prion diseases, the spread of these proteins from cell to cell and within the nervous system seem to follow similar patterns. There is no evidence that other neurodegenerative diseases such as Alzheimer’s or Parkinson’s disease are contagious. The similarity in the underlying mechanisms of disease progression raises common fundamental research questions and suggests future approaches for prevention, diagnosis and therapy.
Prof. Charles Weissmann (born in 1931) is a Professor Emeritus at the Scripps Research Institute in Florida (USA). The Swiss molecular biologist studied medicine in Zurich and obtained his doctorate in organic chemistry. He completed a postdoctoral fellowship at the School of Medicine of the New York University and subsequently joined the faculty there. In 1967, he returned to the University of Zurich where he assumed the position of Director of the Institute for Molecular Biology. In 1999 he accepted a position with the MRC Prion Unit at the University College London. Five years later he was appointed to the new Scripps Research Institute Florida, where he established and directed the Institute for Infectiology for eight years. Website: http://www.scripps.edu/florida/research/faculty/weissmann
Prof. Adriano Aguzzi (born in 1960) was appointed Director of the Swiss National Reference Center for Prion Diseases at the end of 1995 and has been the Director and a member of the faculty of the Institute for Neuropathology at the University of Zurich since 1997. He obtained his medical degrees in Fribourg and Basel. Following periods at Columbia University, the University of Zurich and the Research Institute for Molecular Pathology in Vienna, he has held the position of Medical Director and Private Lecturer in the fields of pathology and neuropathology at the University of Zurich since 1993. Aguzzi is a citizen of both, Italy and Switzerland. Website: http://www.neuroscience.ethz.ch/research/aging_disorders/aguzziAbout Piepenbrock
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