THE ETIOLOGY AND PATHOGENESIS OF HUNTINGT ONS-DISEASE - RECENT ADVANCES

Huntingtons's disease (HD) is a neurodegenerative disorder for which n o causal therapy is currently available. It is inherited as an autosom al dominant trait and the responsible gene was localized 10 years ago to chromosome 4. Recently the gene has been identified. This discovery now allows a definitive diagnosis to be made in most cases. Pre-clini cal testing, however, requires careful psychological counselling. So f ar the structure and function of the gene product are unknown. Using e xperimental neurotoxicological methods it is possible to mimic several features of the disease process to such an extent that preliminary hy potheses regarding the function of the encoded protein are possible. I t will be of great interest to see how the results of molecular geneti c studies and of experimental research can be synthesized in the futur e.