1998 Award Winner

Professor Dr. Michael Wegner
Center of Molecular Neurobiology, University Hospital (UKE) in Eppendorf, Hamburg, Germany."To my mind, this type of award is extremely important, as winning a prestigious international prize can make a big difference to a scientist at a fairly early stage in his or her career." ( Michael Wegner)

The work of Michael Wegner
Dr. Wegner and his group carry out research into those transcription factors which regulate the generation of cell diversity in the nervous system. The nervous system is a highly complex structure. Its function requires the presence of numerous different cell types which are specialized to perform distinct tasks. Generation of all these cell types from an originally homogeneous pool of cells during development is a complex process that has to be tightly controlled. One of the groups of proteins involved in this control are the so-called transcription factors, which determine whether a certain gene is active or inactive in a given cell at a given time. One of the transcription factors identified by Dr. Wegner is the Sox10 protein. Sox10 is present in the organism from an early point in the development onwards. Sox10 can already be found in a group of cells of the early embryo, from which the so-called peripheral nervous system is formed. This part of the nervous system serves to innervate the organs and to connect them to the brain. Dr. Wegner showed that the Sox10 gene is altered in a spontaneous mouse mutant. With both copies of the gene altered, these mice embryos die before birth. With only one copy altered, mice are viable, but are lacking part of their peripheral nervous system. Specifically, the hind region of the intestine fails to be innervated, so that its contents cannot be properly cleared and remain trapped, resulting in severe constipation and a heavily dilated colon. Similar symptoms are also observed in children suffering from Hirschsprung disease, a congenital defect that affects 1 in 5,000 live births. Sox10 alterations were also detected in patients with Hirschsprung disease. Interestingly, all of these patients also exhibited deafness and pigmentation defects of skin, hair or eyes. All detected alterations of the Sox10 gene led to Sox10 proteins that are defective in one or more of the functions vital to a transcription factor, and are therefore inactive. These results show that the scientific approach chosen by Dr. Wegner will help to clarify the origins of genetic diseases in humans. Furthermore, it may be useful for the development of therapeutic strategies for neurodegenerative diseases such as Multiple Sclerosis, Parkinson's disease and Alzheimer's disease.

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