1996 Award Winner
Professor Dr. Ueli Suter
Institute of Cell Biology, Swiss Federal Institute of Technology, ETH Zürich, Switzerland."This fine encouragement has played a key role in fostering my motivation to continue and accelerate my research in basic and applied projects."
The work of Professor Suter
Professor Suter's research focuses on the molecular and cellular basis of the development and regeneration of the nervous system of vertebrates and on the associated neuro-degenerative diseases in humans. Major emphasis is placed on research into the molecular nervous sheath, the technical term for which is myelin. This structure is essential for the nervous system as myelin insulates nerve fibres (in the same way as plas-tic insulates an electrical cable), thus enabling electrical control signals to be transmitted efficiently over long distances (e.g. from spinal cord to the hands and feet). It is therefore no surprise that a number of human diseases - the most well-known of which are multiple sclerosis and hereditary neuropathies - are caused by damage to the myelin layer. Together with colleagues in the USA, Professor Suter's research group succeeded in identifying a specific gene which, in its most frequently-occurring form, is mutated in human neuropathies which are predominantly hereditary. This disease, which affects approximately one in 4,000 people, is known as Charcot-Marie-Tooth syndrome (after the people who discovered it) and is characterized primarily by the destruction of the myelin layer of peripheral nerves. Consequently, the transmission of electrical signals in these patients is severely impaired. In slight cases, this condition causes partial numbness and muscular weaknesses in the hands and feet, whereas a severe case can destroy all muscular functions and confine the sufferer to a wheelchair. The researchers in Professor Suter's group were able to prove that the defective gene responsible for these conditions, which was given the name PMP22, can be altered by various genetic mechanisms which can be correlated with the severity of the disease under observation. These discoveries have enabled this common hereditary disease to be detected using molecular biological methods in clinics. This means that it is possible not only to give the sufferer a definite diagnosis, but also to avoid taking nerve biopsies, which is often necessary in such cases. The scientists in Professor Suter's group continued their investigations by producing a series of transgenic mouse and rat models for the aforementioned group of hereditary neuropathies. There is now a very good chance that these animal models will not only reveal important information on the exact mechanisms of the disease, but could also be a major contributory factor in developing suitable therapies for these currently untreatable conditions.