DEGENERATIVE ATAXIAS - GENETICS, PATHOGENESIS AND ANIMAL-MODELS

At present, seven inherited neurodegenerative disorders have been iden tified to be caused by the polyglutamine-coding CAG triplet expansions in the genes responsible. The long stretch of polyglutamines appears to play a key role in the pathogenesis. Disease models, just recently established with cultured cells and animals, will enable elucidation o f common disease mechanisms and treatments.