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

Abstract:

: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.

journal_name

Neuroscience

journal_title

Neuroscience

authors

Butterworth NJ,Williams L,Bullock JY,Love DR,Faull RL,Dragunow M

doi

10.1016/s0306-4522(98)00129-8

subject

Has Abstract

pub_date

1998-11-01 00:00:00

pages

49-53

issue

1

eissn

0306-4522

issn

1873-7544

pii

S0306452298001298

journal_volume

87

pub_type

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