期刊
NEUROBIOLOGY OF DISEASE
卷 15, 期 3, 页码 667-675出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2003.12.010
关键词
Huntington's disease; 3-Nitropropionic acid; kainate receptor GluR6 knockout mice; age at onset disease history
Age of onset of Huntington's disease (HD) statistically correlates with the length of expanded CAG repeats in the IT15 gene. However, other factors such as polymorphism in the 3' untranslated region of the GluR6 kainate receptor gene subunit may contribute to variability in the age at onset. To investigate this issue, we studied the motor disorder and related striatal damage induced by 3-nitropropionic acid (3-NP) subacute administration in GluR6 knockout mice (GluR6(-/-)) as compared to wild-type mice. In two different age groups (6 months and I year), we observed that GluR6(-/-) mice did not display more motor impairment nor more striatal histopathological damage than GluR6(+/+) mice, although 1-year-old GluR6(-/-) mice displayed reduced activity parameters either at baseline or after 3-NP administration compared to GluR6(+/+). In both age groups, Glu6(-/-) mice died earlier and displayed earlier motor symptoms during 3-NP-induced metabolic compromise, suggesting that GluR6-containing kainate receptors may be implicated during neurodegeneration, such as in HD, rather than in the final outcome. (C) 2004 Elsevier Inc. All rights reserved.
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