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Trinucleotide repeat length and rate of progression of Huntington's disease

✍ Scribed by Dr. Sergei N. Illarioshkin; Shuichi Igarashi; Osamu Onodera; Elena D. Markova; Natalya N. Nikolskaya; Hajime Tanaka; Tina Z. Chabrashwili; Nina G. Insarova; Kotaro Endo; Irina A. Ivanova-Smolenskaya; Shoji Tsuji

Publisher: John Wiley and Sons
Year: 1994
Tongue: English
Weight: 613 KB
Volume: 36
Category: Article
ISSN: 0364-5134
DOI: 10.1002/ana.410360412

No coin nor oath required. For personal study only.

✦ Synopsis

The Huntington's disease gene contains an expanded unstable (CAG), repeat, and the repeat lengths have been shown to correlate with the age of onset. Using detailed clinical scales, we evaluated the rate of progression 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 psychiatric) 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.

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