TRINUCLEOTIDE REPEAT LENGTH AND RATE OF PROGRESSION OF HUNTINGTONS-DISEASE

The Huntington's disease gene contains an expanded unstable (CAG)(n) r epeat, and the repeat lengths have been shown to correlate with the ag e of onset. Using detailed clinical scales, we evaluated the rate of p rogression of Huntington's disease and its relationship to the number of triplet repeats. We found significant positive correlation between the rate of progression of clinical symptoms (both neurological and ps ychiatric) and CAG repeat length. These data suggest an important role of expanded trinucleotide repeat length in affecting the pathological process during the entire course of Huntington's disease.