Trinucleotide (CAG) repeat length is positively correlated with the degree of DNA fragmentation in Huntington's disease striatum

被引：83

作者：

Butterworth, NJ

Williams, L

Bullock, JY

Love, DR

Faull, RLM

Dragunow, M ^{[1
]}

机构：

[1] Univ Auckland, Dept Pharmacol, Auckland 1, New Zealand

[2] Univ Auckland, Dept Clin Pharmacol, Auckland 1, New Zealand

[3] Univ Auckland, Dept Anat, Auckland 1, New Zealand

[4] Univ Auckland, Sch Biol Sci, Auckland 1, New Zealand

来源：

NEUROSCIENCE | 1998年 / 87卷 / 01期

关键词：

DNA fragmentation; apoptosis; Huntington's disease; CAG repeats;

D O I：

10.1016/S0306-4522(98)00129-8

中图分类号：

Q189 [神经科学];

学科分类号：

071006 [神经生物学];

摘要：

Recent studies using DNA fragmentation assays suggest a role for apoptosis in cell death in Huntington's disease. In this study, we investigated the relationship between the degree of DNA fragmentation and the number of trinucleotide (CAG) repeats of the Huntington's disease gene in striatal tissue from Huntington's disease brains. We used frozen striatal tissue from 27 post mortem Huntington's disease brains (graded 0-4 on the Vonsattel classification, post mortem delay ranging from 4 to 41 h), plus control sections which were age, sex and post mortem delay matched from neurologically normal and Alzheimer's diseased striatal tissue. Our results show a significant positive correlation between the number of CAG repeats in the Huntington's disease gene and the degree of DNA fragmentation in Huntington's disease striatum. These results suggest that expanded CAG repeats in the Huntington's disease gene may lead to neuronal degeneration in Huntington's disease through an apoptotic mechanism. (C) 1998 IBRO. Published by Elsevier Science Ltd.

引用

页码：49 / 53

页数：5

共 22 条

[1]

THE RELATIONSHIP BETWEEN TRINUCLEOTIDE (CAG) REPEAT LENGTH AND CLINICAL-FEATURES OF HUNTINGTONS-DISEASE [J].