Massimo Zeviani awarded the Grand Prix of the NRJ Foundation in Paris

On Wednesday, 5 June 2013, Professor Massimo Zeviani will be presented with the 2013 Grand Prix of the NRJ Foundation, on "Genetics of degenerative diseases", under the cupola of the Institute of France. The award is made to support Professor Zeviani’s work on the genetics of mitochondrial diseases. "The Prize of the NRJ Foundation will allow me to continue my research which aims at understanding why mitochondrial deficiency leads to brain damage and how to correct or even halt the progression of disease in patients." - Massimo Zeviani

The NRJ Foundation

The NRJ Foundation was created by Jean-Paul Baudecroux, President of NRJ Group in May 1999 under the auspices of the Institute of France, for humanitarian purposes and to support researchers. Its purpose is to advance medical research in neuroscience. The Foundation awards an annual prize of 100,000 Euros to reward and encourage scientific work carried out by an individual or a group of people in a public or private institution. Through these grants, and total freedom in the choice of beneficiaries, the NRJ Foundation can achieve its goal of promoting scientific research and thus relieve those who suffer, are isolated, and vulnerable due to pain. The scientific jury selects the best records of scientists in two calls for applications per year, one for the scientific prize of 100,000 Euros and one for grants of 40,000 Euros each. Since 1999, the NRJ Foundation has awarded an annual science prize on a different theme and the amount of 100,000 Euros to the researcher who, in the eyes of its Scientific Committee, contributed most to the progress of neuroscience research. Previous Grand Prix Recipients are listed on the NRJ Foundation website.

The Institute of France

Established in 1795 to promote the Arts, Sciences and Humanities, the Institute of France groups together five academies: Académie française, Académie des inscriptions et belles-lettres, Académie des sciences, Académie des beaux-arts, Académie des sciences morales et politiques. Meanwhile, it supports foundations, created by individuals or companies to whom it provides its expertise and know-how, and a guarantee of continuity. The Institute also owns an important artistic heritage consisting of houses and unique collections that have been handed down since the late nineteenth century, including: the Château de Chantilly, the Musée Jacquemart-André, the Royal Abbey of Chaalis, the Château de Langeais, the Manoir de Kerazan and the Villa Kérylos.

Professor Massimo Zeviani

Director of the MRC Mitochondrial Biology Unit in Cambridge, UK, Professor Massimo Zeviani founded the Department of Molecular Neurogenetics at the Besta Institute in Milan. After his graduation in Medicine and specialization in Endocrinology at the University of Padua, Italy, he began his research into mitochondrial diseases at the Neurological Institute of Columbia University in New York, before undertaking post-graduate study in Neurology at the University of Verona in 1989 and his doctorate in genetics at the University of Paris V in 1996. His research focuses on the genetics of mitochondrial diseases, which mainly affect the nervous system and skeletal muscles. The extraordinary genetic and biochemical complexity of mitochondria is a formidable hurdle to develop effective treatments for these diseases. Professor Zeviani has strived to blend medical research on patients with basic science to advance the molecular interpretation of mitochondrial diseases. In turn, research on mitochondrial disease genes has contributed essential information to the understanding of the biology of mitochondria. Professor Zeviani is also committed to establishing a translational agenda for the development of effective therapeutic strategies based on activation of mitochondrial biogenesis, targeting of therapeutic genes, and modulation of mitochondrial autophagy and apoptosis in critical organs.

Principal results

The principal results of Professor Zeviani's work concern the elucidation of molecular mechanisms linking mitochondrial dysfunction to neurodegeneration, with the ultimate goal of developing effective therapies. In New York, he established that the sporadic mitochondrial disease Kearns-Sayre syndrome (KSS) was specifically associated with deletions of mitochondrial DNA. He then identified with his team numerous genes primarily responsible for a wide spectrum of disorders related to mitochondrial dysfunction. He also participated in the identification of several genes and proteins responsible for hereditary neurodegenerative diseases, including transthyretin, responsible for familial amyloidotic polyneuropathy, SMN1, responsible for spinal amyotrophy, and paraplegin, responsible for a common form of hereditary spastic paraplegia. While this work has continued to generate new discoveries regarding the molecular basis of mitochondrial dysfunction, more recently Professor Zeviani has developed a program of experimental therapies in models of mitochondrial disease whose results will soon be transferred to clinical trials in patients.